MINI REVIEW article

Front. Endocrinol., 18 April 2018

Sec. Cancer Endocrinology

Volume 9 - 2018 | https://doi.org/10.3389/fendo.2018.00177

Deoxyribonucleotide Triphosphate Metabolism in Cancer and Metabolic Disease

  • RB

    Raquel Buj

  • KM

    Katherine M. Aird *

  • Department of Cellular and Molecular Physiology, Penn State College of Medicine, Hershey, PA, United States

Article metrics

View details

72

Citations

13,5k

Views

3,3k

Downloads

Abstract

The maintenance of a healthy deoxyribonucleotide triphosphate (dNTP) pool is critical for the proper replication and repair of both nuclear and mitochondrial DNA. Temporal, spatial, and ratio imbalances of the four dNTPs have been shown to have a mutagenic and cytotoxic effect. It is, therefore, essential for cell homeostasis to maintain the balance between the processes of dNTP biosynthesis and degradation. Multiple oncogenic signaling pathways, such as c-Myc, p53, and mTORC1 feed into dNTP metabolism, and there is a clear role for dNTP imbalances in cancer initiation and progression. Additionally, multiple chemotherapeutics target these pathways to inhibit nucleotide synthesis. Less is understood about the role for dNTP levels in metabolic disorders and syndromes and whether alterations in dNTP levels change cancer incidence in these patients. For instance, while deficiencies in some metabolic pathways known to play a role in nucleotide synthesis are pro-tumorigenic (e.g., p53 mutations), others confer an advantage against the onset of cancer (G6PD). More recent evidence indicates that there are changes in nucleotide metabolism in diabetes, obesity, and insulin resistance; however, whether these changes play a mechanistic role is unclear. In this review, we will address the complex network of metabolic pathways, whereby cells can fuel dNTP biosynthesis and catabolism in cancer, and we will discuss the potential role for this pathway in metabolic disease.

Introduction

The maintenance of deoxyribonucleotide triphosphate (dNTP) pools is critical for multiple cellular pathways. For instance, imbalances in dNTPs are associated with genomic instability (1). Likewise, they have also been shown to disturb mitochondrial DNA (mtDNA) and consequently mitochondrial fitness, which may lead to mitochondrial diseases (MDs), such as diabetes, obesity, and cancer (2). Additionally, disorders of purine and pyrimidine metabolism (DPPM) profoundly affect cell metabolism, underlying the importance of nucleotides for cell behavior (3). Thus, both nucleotide synthesis and degradation must be exquisitely fine-tuned. In this review, we will focus on synthesis of dNTPs and the consequences of dNTP pool imbalances in cancer and MDs.

Healthy dNTP Pools

A correct balance of dNTPs is necessary for the prevention of multiple pathologies. A healthy cell must maintain two asymmetric and spatial-temporal dNTP pools; one for nuclear DNA synthesis and repair and another for mtDNA replication and repair. Disruptions in dNTP balance are associated with enhanced mutagenesis, leading to genomic instability, which promotes cancer (4), and may have a role in metabolic disease (5).

Cytosolic dNTP pool concentrations positively correlate with the cell cycle. In fact, the amount of dNTPs at the beginning of S-phase is not enough for a complete DNA duplication (6). The S-phase increase in dNTPs is necessary for faithful nuclear DNA replication. mtDNA is replicated continuously in post-mitotic cells, and faithful maintenance of mtDNA also depends on correctly balanced dNTPs (7). Thus, both proliferating and non-proliferating cells need to fine-tune nucleotide and dNTP synthesis to allow for both nuclear and mtDNA replication and repair to maintain the health of the cell.

Anabolism and Catabolism of Nucleotides

Cells possess two biosynthetic pathways to produce dNTPs: de novo and salvage (8). Purines and pyrimidines arise from two different de novo pathways that generate nucleotides starting from raw material (glucose, glutamine, aspartate, and HCO3) (9). The de novo nucleotide synthesis pathway is highly energy-intensive (9). Therefore, cells have developed a more energy-efficient route to synthesize nucleotides, termed the salvage pathway (10). The salvage pathway acts as a recycling plant taking free nitrogen bases and nucleosides arising from nucleic acid breakdown and diet (9). Nucleosides are hydrophilic compounds, thus proper function of nucleoside transporters (SLC29 and SLC28 families) is an essential requirement for salvage pathway function (11). Ribonucleotides obtained by either pathway can be reduced to their deoxyribonucleotide counterpart in a reaction catalyzed by ribonucleotide reductase (RNR) (12).

Turnover of RNA and other nucleotides occurs regularly to maintain homeostasis. Human cells cannot break down the purine ring. Purine catabolism involves a sequence of three reactions in which nucleotides are stripped step-by-step from their phosphates and sugar to finally become oxidized to the end product uric acid (UA), which is excreted into the urine (13). Conversely, uracil and thymidine rings can be completely degraded to β-alanine and β-aminoisobutyrate, respectively. Subsequently, both metabolites can be excreted or transformed into intermediates of the tricarboxylic acid (TCA) cycle (14). Biosynthesis and catabolism of nucleotides and dNTPs are highlighted in Figure 1.

Figure 1

Impaired Nucleotide Metabolism in Cancer and Metabolic Disease

Deregulation of nucleotide metabolism is associated with a broad spectrum of pathological conditions, including cancer and MDs (15–17). Virtually all metabolic pathways have been implicated in dNTP biosynthesis. Thus, de novo and salvage pathways, as well as all involved anapleurotic reactions (Figure 1), need to be highly cross-regulated.

It is well known that cancer cells must increase dNTP biosynthesis (18) to ensure rapid replication of the genome (17). This occurs through a variety of pathways (discussed below). In contrast, MDs are caused by congenital or acquired genetic defects in metabolic enzymes. DPPM are due to abnormalities in the biosynthesis, interconversion, and degradation of nucleotides (19). DPPM have a wide variety of clinical presentations, highlighting the importance of proper nucleotide metabolism for cell and organism function (15). Alterations in nucleotide metabolism are also present in other metabolic-related pathological conditions, such as diabetes, obesity, and insulin resistance (20–22) (Table 1). In this section, we will summarize some important features affecting nucleotide metabolism in cancer and MDs.

Table 1

Gene/family/pathwayKnown role in dNTP metabolismExpression in cancerExpression in metabolic disease
Purine/pyrimidine synthesis pathwayNecessary for de novo dNTP biosynthesis (8)aIncreased (23) or mutated (24, 25)Heptatic steatosis (uridine metabolism) (↓) (26)Diabetesb (↓) (27)
MTORPromotes glucose uptake (28); promotes de novo nucleotide biosynthesis (29, 30)Increased (31)Diabetes (↑) (32)Obesity (↑) (33)
MYCInduces glucose uptake and utilization (34); transcriptionally regulates nucleotide metabolic enzymes (23, 35)Increased (oncogene) (23)Insulin resistance (↑)cObesity (↑)c (36)
TP53Negative regulator of pentose phosphate pathway through G6PD (37); gain-of-function mutations increase gene transcription of genes for dNTP synthesis (38)Decreased or mutated (tumor suppressor) (39)Insulin resistance (↑)Glucose intolerance (mut) (5)Mitochondrial changes (mut)d (40)
PI3K-AKT pathwayOncogenic activation promotes glucose and glutamine uptake and catabolism (41)Increased (oncogenes) (41)Diabetes (↑) (42)Nonalcoholic fatty liver disease (↑) (43)Obesity (↑) (44)
ERK-MAPK pathwayRegulation of CPS II in de novo pyrimidine synthesis (45)Increased (oncogenes) (46)Diabetes (↑) (47)Obesity (↑) (48)
G6PDRate-limiting for ribose-5-phosphate synthesis from the PPP (49)aIncreased or mutated (50)Obesity (↑) (51)Diabetes (↑) (52)
RRM1Catalytic subunit of the ribonucleotide reductase (RNR); catalyzes the reduction of deoxyribonucleotides from ribonucleotides (12)aIncreased or decreased (53)Unknown
RRM2Regulatory subunit of RNR (12); S-phase regulated (54); rate-limiting enzyme in the reduction of deoxyribonucleotides from ribonucleotides (55)aIncreased (oncogene) (53)Unknown
RRM2BRegulatory subunit of the RNR (56); formation of deoxyribonucleotides from ribonucleotides for DNA damage repair and mitochondrial DNA (mtDNA) replication (57–59)aIncreased or decreased (53)Mitochondrial disorders (↓) (60)
SLC25 familyMitochondrial nucleoside transporters (61)Important for mtDNA pools through the salvage pathway (62)Increased (63)Mitochondrial disease (mut)eMitochondrial dysfunction (↓)f (61)
SLC29 and SLC28 familiesNucleoside transporters that are important for the salvage pathway (11, 64, 65)Increased (11)Diabetes (mut)g (66)
TK2Phosphorylates deoxycytidine to generate dCTP (67)UnknownMitochondrial disease (↓) (68)
DGUOKCatalyzes the conversion of deoxyguanosine to dGMP (67)aMutatedh (69, 70)Mitochondrial disease (mut) (69)
TWNKMitochondrial helicase (71)UnknownMitochondrial dysfunction (mut) (72, 73)
POLGCatalytic subunit of the mitochondrial DNA polymerase (74)Mutated (75–77)Mitochondrial disease (mut) (77–79)
Ataxia-telangiectasia mutatedIncreases glucose/glutamine uptake and inhibits the PPP (80)Mutated (81)Mitochondrial dysfunction (mut)iInsulin resistance (mut)i (82)
XORCatalyzes the conversion of xanthine to uric acid (83)aIncreasedj or decreased (84)Metabolic syndrome (mut)kInsulin resistance (mut)kDiabetes (mut)kFatty liver disease (mut)k (85)

Genes, protein families, and pathways discussed in this review: role in deoxyribonucleotide triphosphate (dNTP) metabolism and expression in cancer and metabolic disease.

aThese genes/pathways are shown in Figure 1.

bThese studies show that purines and pyrimidines are downregulated in diabetes. It is not known whether changes in purine or pyrimidine synthesis genes are the mechanism behind this observation.

cIncreased MYC expression counteracts insulin resistance and obesity.

dOccurs in patients with Li–Fraumeni syndrome.

eSLC25A4 (86).

fSLC25A33 and SLC25A36 have only been tested in mouse models (87, 88).

gSLC29A3 is the only gene in this family that has been found to affect metabolic disease.

hWhile the data are limited, some patients with DGUOK mutations have hepatocellular carcinoma.

iOccurs in patients with ataxia-telangiectasia.

jIncreased XOR expression/activity is likely important for cancer initiation; however, XOR expression is decreased in most established tumors.

kOccurs in patients with XOR deficiency.

Deregulation of Major Growth Signaling Pathways Leads to Nucleotide Pool Imbalances in Cancer and Metabolic Disease

The main growth signaling pathways (PI3K-AKT and ERK1/2-MAPK) are induced and maintained during metabolic reprogramming of cancer (18). Additionally, deregulation of these pathways may contribute to different MDs, including diabetes, obesity, or steatosis resistance (33, 89, 90). These pathways sense and orchestrate nutrient utilization; therefore, is not surprising that alterations in these pathways affect energy and biomass production and cause a broad variety of diseases.

mTOR is a central signaling pathway that integrates environmental inputs (e.g., nutrients and hormones) into downstream pathways to control many cellular processes (91). This includes regulation of metabolism, growth, and survival (32). Indeed, the mTORC1/2 pathway not only promotes glucose uptake and protein and lipid biosynthesis, but also promotes nucleotide biosynthesis (29, 30) and uptake of nucleosides through transporters (88). At least one member of this pathway is altered in 38% of human cancer (92). Altered metabolism induced by aberrant mTORC1 activation has also been shown to play a role in diabetes and obesity (32, 93).

c-Myc, one of the most commonly altered proteins in human cancer, is also regulated by PI3K-AKT and ERK1/2-MAPK pathways (94). c-Myc is a highly pleiotropic transcription factor considered a master regulator of cell metabolism (34, 35) through regulation of glycolysis, glutamine metabolism, and mitochondrial biogenesis (95, 96). Indeed, c-Myc has been shown to induce hepatic glucose uptake and utilization, while blocking gluconeogenesis and ketogenesis, suggesting a counteracting effect of c-Myc in obesity and insulin resistance (36, 97). In addition to regulating glucose and glutamine, substrates for purine and pyrimidine biosynthesis (Figure 1) (98), c-Myc also transcriptionally regulates nucleotide metabolic enzyme gene expression (35). Thus, deregulation in c-Myc acutely alters nucleotide homeostasis in cancer (99), and it is interesting to speculate that the role of c-Myc in MDs is also related to nucleotide metabolism.

Previous publications from our laboratory and others have shown that DNA damage and DNA damage response (DDR) proteins regulate dNTP biosynthesis in the context of cancer (80, 100, 101). Interestingly, upregulation of p53, a key player in the DDR, in adipose tissue is associated with increased inflammation and insulin resistance (102). Notably, wild-type p53 negatively regulates G6PD activity (37), the rate-limiting enzyme of the pentose phosphate pathway and one of the most important sources of nucleotides (103). Upregulation of G6PD correlates with functional defects in liver, heart, and pancreas of obese and diabetic animals (104). Although the relationship between G6PD upregulation and increased oxidative stress has been studied in MD (105), the implication for nucleotide metabolism has not yet been addressed. More research is needed to understand the contribution of dNTP imbalances due to G6PD deregulation in diabetes and obesity.

An imbalance in nucleotides has been shown in two different studies related to diabetes (106, 107). Additionally, pyrimidine metabolism has been linked to fatty liver (26). Interestingly, increasing evidence suggests a link between obesity, a risk factor for non-alcoholic fatty liver disease (108), and cancer. Obese patients show many cancer-promoting features, such as chronic low-level inflammation (109), insulin-resistance/diabetes (110), and deregulation of mTORC1 (111). Although the contribution of deregulated nucleotide pools promoting cancer has been extensively demonstrated (18, 112–115), their role in MD and metabolic-related diseases has not yet been elucidated. Based on these recent studies, we speculate that deregulation of nucleotide pools may in part contribute to the altered metabolic landscape promoting obesity and diabetes. Studying the implications of altered nucleotide pools in these diseases would open a therapeutic window based on modulation of nucleotide metabolism.

RNR in Cancer and Metabolic Disease

Ribonucleotide reductase reduces ribonucleotides to the corresponding deoxyribonucleotides (116, 117). In mammals, RNR is a tetrameric enzyme composed of two homodimeric subunits, RRM1 and RRM2. Whereas, RRM1 is continuously expressed throughout the cell-cycle, expression of RRM2 is activated upon entry into S-phase (54, 118). Additionally, RRM2 is rapidly degraded via the proteasome in G2 (12, 119). Thus, RRM2 is considered rate-limiting for RNR activity. RRM2B (RNR subunit M2B) is an alternative M2 subunit that is induced by p53 activation in response to DNA damage (56). RRM2B is not cell-cycle regulated per se, but it plays key roles in enhancing dNTP synthesis in cells under stress (120–122) and mediating mtDNA synthesis and repair (57–59).

The role of RNR in cancer is clear as it was one of the first identified DNA damage-induced enzymes (123). While RRM2 overexpression is tumorigenic, leading to lung neoplasms in vivo, RRM1 reduces tumor formation, migration, and metastasis [reviewed in Ref. (53)]. Previous studies from our lab and others have shown the potential of RRM2 as a prognostic and diagnostic biomarker in multiple cancers (112, 124–127). However, the utility of RRM1 and RRM2B as a tumor biomarker is still unclear [reviewed in Ref. (53)].

Although there is no study directly linking RNR with MD, RRM2B is required for mtDNA synthesis and healthy mitochondrial function (57). Deregulated mitochondria are associated with a higher risk of diabetes and obesity (discussed below). Therefore, it is possible that RNR function is linked to these MDs (Table 1). More mechanistic studies will be needed to determine the role for RNR in obesity and diabetes.

Mitochondrial Dysfunction in dNTP Pool Disruption During Cancer and Metabolic Disease

The mitochondria are one of the most important organelles for eukaryotic function (128). In addition to the production of ATP through oxidative phosphorylation, mitochondria are also the scaffold of several metabolic reactions for cellular building block synthesis (e.g., fatty acid beta-oxidation, one-carbon/folate cycle, TCA cycle, amino acid metabolism, etc.) (129). Hence, altered mitochondrial behavior has a broad impact on cellular metabolism.

Maintenance of mitochondrial dNTP pools is critical for proper mtDNA function. Alterations in nuclear genes involved in transport of cytosolic dNTPs (e.g., SLC25A4), the salvage nucleotide biosynthesis in the inner mitochondrial membrane (e.g., TK2 and DGUOK), and genes involved in mtDNA replication (e.g., TWNK and POLG) are implicated in both cancer and metabolic syndromes (63, 68, 77–79, 130–133). Moreover, dysfunction in the electron transport chain induces oxidative stress, which has been associated with impaired one-carbon metabolism (134, 135), an essential anapleurotic pathway for both purine and pyrimidine nucleotides. Mitochondrial genomic instability due to increased levels of reactive oxygen species (ROS) and/or mutations in mtDNA or nuclear genes involved in mitochondria function are underlying factors of MDs, and contribute to cancer and diabetes (136). Alterations in genes discussed above that are important for dNTP homeostasis and mitochondrial function are highlighted in Table 1.

Although the link between mitochondrial dysfunction and MD has been studied for the past two decades, the results are contradictory (137). These contradictory results mainly arise from the complex relationship between mitochondria and metabolism, but also from the lack of global and standardized methodological strategies to phenotype insulin-resistance in humans (138). Dysregulation of nucleotide metabolism is an important aspect of mitochondrial dysfunction; therefore, their role in MDs should not be ignored.

Relationship Between DPPM and Cancer

It is clear that cancer is a metabolic disease; however, a predisposition to cancer is not a foregone conclusion in patients with DPPM, who by definition have alterations in nucleotide supplies. Interestingly, while deficiencies in some metabolic pathways known to play a role in nucleotide synthesis are pro-tumorigenic, others confer an advantage against the onset of cancer. This highlights the large variability in the clinical presentation of these disorders.

Alterations in p53 or ataxia-telangiectasia mutated (ATM) lead to metabolic changes and predispose patients to cancer. Patients with germline TP53 (encoding for p53) mutations have Li–Fraumeni syndrome and are predisposed to cancer (139, 140). Interestingly, a recent report showed that nucleotide metabolism is regulated by the gain-of-function activity of mutant p53 (38). Consistently, wild-type p53 negatively regulates G6PD and PPP activity to decrease dNTP synthesis (37). Similarly, our group has previously shown that ATM (mutated in some ataxia-telangiectasia patients) inactivation increases glucose uptake and enhances glucose flux through the PPP and ultimately increases dNTP biosynthesis (Figure 1) (80, 141). Indeed, patients with ATM mutations show alterations in glucose homeostasis (142, 143). It is well-known that these patients have an increased susceptibility to cancer (144). It is interesting to speculate that alterations in dNTP metabolism may play a role in the cancer predisposition in these patients; however, further studies are needed to support this notion.

Other DPPM confer a tumor suppressive benefit. For instance, patients with G6PD deficiency have a reduced risk of some cancers (145–147) (Table 1). This suggests that hyperactivity of dNTP synthesis is more likely to increase cancer risk than deficiencies in synthesis.

Finally, some DPPM have both a pro- and anti-tumorigenic effect. Deficiency in xanthine oxidoreductase (XOR), the enzyme that catalyzes the last step in purine catabolism (Figure 1), increases UA (148). There is a dual role for UA in cancer, the so-called the oxidant–antioxidant UA paradox (149). On one hand, extracellular UA is a potent ROS scavenger, thus protecting cells against oxidative stress (150). On the other hand, high intracellular levels of UA in a XOR-deficient cellular background promote dNTP biosynthesis and tumor growth by shuttling XOR precursors (xanthine and hypoxanthine) into the purine salvage pathway (149). Additionally, intracellular UA is pro-inflammatory by inducing NADPH-oxidases that lead to oxidative stress and cancer (151, 152). This again emphasizes the complex nature of these disorders in relation to cancer (Table 1).

Together, the lack of consensus in predisposition to cancer in DPPM patients points to the significant redundancy in the dNTP biosynthetic pathways. This should not be surprising due to the fact that dNTP synthesis is critical for organismal survival and, therefore, we have evolved to have multiple metabolic arms feeding into the same pathway. Understanding whether these patients are predisposed or not to cancer will be incredibly important for the clinical management of these patients.

Therapeutic Modulation of Deoxyribonucleotide Metabolism in Cancer and Metabolic Disease

As described in this review, the balance of dNTPs must be tightly regulated in the cell. Many cancer types show alterations in dNTP levels, supporting their rapid proliferation. Likewise, defective mutations in anabolic and catabolic nucleotide enzymes, causing imbalances in the dNTP pools or in their precursors, are associated with different grades of disease severity in DPPM. Thus, it is not surprising that therapies for both cancer and DPPM focus on restoration of the normal balance of intracellular nucleotides.

Some of the first chemotherapeutic agents were cytotoxic nucleoside analogs and nucleobases (e.g., thiopurines and fluoropyrimidines) (153). These antimetabolites have a similar molecular structure to endogenous nucleotides and interfere with nucleotide metabolic pathways and DNA/RNA synthesis (154). Inhibitors of RNR were one of the first cancer therapies [reviewed in Ref. (53)] and are still used today. For instance, gemcitabine, a chemotherapeutic nucleoside analog, is used in pancreatic adenocarcinoma, but also in breast, bladder, and non-small cell lung cancer (155). Unfortunately, resistance to gemcitabine in common, often through an increase in nucleotide synthesis pathways or transport of nucleosides (156). Other successful chemotherapeutic regimens include methotrexate, which reduces substrates for purine and pyrimidine biosynthesis (157). Finally, specific inhibition of enzymes in the de novo pathway and/or in anapleurotic reactions (glucose and glutamine metabolism) has also been used as adjuvant therapies in cancer (154).

The spectrum of nucleotide therapies for DPPM is much broader in scope due to the high variability of deficiencies (3). Thus, deficiencies resulting in the overproduction of UA are treated with allopurinol, an inhibitor of xanthine oxidase (16). In other cases, patients can be treated with oral supplements of specific nucleotides they are lacking (16). What is clear is that cancer patients with DPPM cannot be treated with antimetabolites such as 5-fluoro-uracil due to severe side effects (19). This suggests that cancer patients, DPPM must remain above a certain threshold of nucleotide pools to remain healthy. Finally, no nucleotide therapies are currently used for MDs, such as diabetes or obesity. More studies will need to be performed to determine whether nucleotide metabolism plays a contributing role to these pathologies before these types of therapies can be tested.

Conclusion

For decades researchers and clinicians alike have recognized the importance of fine-tuned dNTP levels for cellular homeostasis, as shown by the number of anti-cancer therapies based on the abolishment of nucleotide synthesis. In addition, the broad range of pathologies associated with congenital defects in nucleotide metabolic enzymes further demonstrates the importance of healthy intracellular dNTP levels. However, the association between cancer and MD and whether nucleotide pools are interconnected in these pathologies remains unclear. Future work will need to focus on mechanistic and population-based studies to determine whether nucleotide pool imbalances in MD lead to changes in cancer predisposition and whether targeting these pathways for cancer therapy affects metabolic homeostasis and function in normal cells.

Statements

Author contributions

RB and KA conceived of and wrote the manuscript.

Acknowledgments

We would like to thank the members of the Aird lab for their thoughtful comments. We would also like to thank Fran Vazquez for help with the dNTP metabolism schematic. RB and KA are supported by an NIH/NCI grant (R00CA194309).

Conflict of interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

References

  • 1

    ZemanMKCimprichKA. Causes and consequences of replication stress. Nat Cell Biol (2014) 16(1):2–9.10.1038/ncb2897

  • 2

    GilkersonR. Commentary: mitochondrial DNA damage and loss in diabetes. Diabetes Metab Res Rev (2016) 32:672–4.10.1002/dmrr.2833

  • 3

    Van Den BergheGVincentMFMarieS. Disorders of purine and pyrimidine metabolism. In: SaudubrayJMBergheGDWalterJH, editors. Inborn Metabolic Diseases: Diagnosis and Treatment. Berlin, Heidelberg: Springer-Verlag (2012). p. 499–518.

  • 4

    MathewsCK. DNA precursor metabolism and genomic stability. FASEB J (2006) 20:1300–14.10.1096/fj.06-5730rev

  • 5

    ShimizuIYoshidaYSudaMMinaminoT. DNA damage response and metabolic disease. Cell Metab (2014) 20:967–77.10.1016/j.cmet.2014.10.008

  • 6

    KohalmiSEGlattkeMMcIntoshEMKunzBA. Mutational specificity of DNA precursor pool imbalances in yeast arising from deoxycytidylate deaminase deficiency or treatment with thymidylate. J Mol Biol (1991) 220:933–46.10.1016/0022-2836(91)90364-C

  • 7

    PaiCCKearseySE. A critical balance: DNTPs and the maintenance of genome stability. Genes (Basel) (2017) 8(2):E57.10.3390/genes8020057

  • 8

    BlakleyRLVitolsE. The control of nucleotide biosynthesis. Annu Rev Biochem (1968) 37:201–24.10.1146/annurev.bi.37.070168.001221

  • 9

    LaneANFanTWM. Regulation of mammalian nucleotide metabolism and biosynthesis. Nucleic Acids Res (2015) 43(4):2466–85.10.1093/nar/gkv047

  • 10

    MoffattBAAshiharaH. Purine and pyrimidine nucleotide synthesis and metabolism. Arab B (2002) 1:e0018.10.1199/tab.0018

  • 11

    YoungJDYaoSYMBaldwinJMCassCEBaldwinSA. The human concentrative and equilibrative nucleoside transporter families, SLC28 and SLC29. Mol Aspects Med (2013) 34:529–47.10.1016/j.mam.2012.05.007

  • 12

    FairmanJWWijerathnaSRAhmadMFXuHNakanoRJhaSet alStructural basis for allosteric regulation of human ribonucleotide reductase by nucleotide-induced oligomerization. Nat Struct Mol Biol (2011) 18:316–22.10.1038/nsmb.2007

  • 13

    MaiuoloJOppedisanoFGratteriSMuscoliCMollaceV. Regulation of uric acid metabolism and excretion. Int J Cardiol (2016) 213:8–14.10.1016/j.ijcard.2015.08.109

  • 14

    WasternackC. Degradation of pyrimidines and pyrimidine analogs-pathways and mutual influences. Pharmacol Ther (1980) 8:629–51.10.1016/0163-7258(80)90079-0

  • 15

    Van GennipAH. Defects in metabolism of purines and pyrimidines. Ned Tijdschr Klin Chem (1999) 24:171–5.

  • 16

    JureckaA. Inborn errors of purine and pyrimidine metabolism. J Inherit Metab Dis (2009) 32:247–63.10.1007/s10545-009-1094-z

  • 17

    KohnkenRKodigepalliKMWuL. Regulation of deoxynucleotide metabolism in cancer: novel mechanisms and therapeutic implications. Mol Cancer (2015) 14:176.10.1186/s12943-015-0446-6

  • 18

    PavlovaNNThompsonCB. The emerging hallmarks of cancer metabolism. Cell Metab (2016) 23:27–47.10.1016/j.cmet.2015.12.006

  • 19

    BakkerJABierauJ. Purine and pyrimidine metabolism: still more to learn. Ned Tijdschr voor Klin Chemie en Lab (2012) 37:3–6.

  • 20

    ChoiY-JYoonYLeeK-YKangY-PLimDKKwonSWet alOrotic acid induces hypertension associated with impaired endothelial nitric oxide synthesis. Toxicol Sci (2015) 144:307–17.10.1093/toxsci/kfv003

  • 21

    KunjaraSSochorMAliMDrakeAGreenbaumALMcLeanP. Pyrimidine nucleotide synthesis in the rat kidney in early diabetes. Biochem Med Metab Biol (1991) 46:215–25.10.1016/0885-4505(91)90069-W

  • 22

    TsushimaYNishizawaHTochinoYNakatsujiHSekimotoRNagaoHet alUric acid secretion from adipose tissue and its increase in obesity. J Biol Chem (2013) 288:27138–49.10.1074/jbc.M113.485094

  • 23

    SatohKYachidaSSugimotoMOshimaMNakagawaTAkamotoSet alGlobal metabolic reprogramming of colorectal cancer occurs at adenoma stage and is induced by MYC. Proc Natl Acad Sci U S A (2017) 114:E7697–706.10.1073/pnas.1710366114

  • 24

    BergMÃ…gesenTHThiis-EvensenEMerokMATeixeiraMRVatnMHet alDistinct high resolution genome profiles of early onset and late onset colorectal cancer integrated with gene expression data identify candidate susceptibility loci. Mol Cancer (2010) 9:100.10.1186/1476-4598-9-100

  • 25

    JoYSOhHRKimMSYooNJLeeSH. Frameshift mutations of OGDH, PPAT and PCCA genes in gastric and colorectal cancers. Neoplasma (2016) 63:681–6.10.4149/neo_2016_504

  • 26

    LeTTZiembaAUrasakiYHayesEBrotmanSPizzornoG. Disruption of uridine homeostasis links liver pyrimidine metabolism to lipid accumulation. J Lipid Res (2013) 54:1044–57.10.1194/jlr.M034249

  • 27

    PillweinKReardonMAJayaramHNNatsumedaYElliottWLFaderanMAet alInsulin regulatory effects on purine- and pyrimidine metabolism in alloxan diabetic rat liver. Padiatr Padol (1988) 23:135–44.

  • 28

    CornuMAlbertVHallMN. MTOR in aging, metabolism, and cancer. Curr Opin Genet Dev (2013) 23(1):53–62.10.1016/j.gde.2012.12.005

  • 29

    Ben-SahraIHoxhajGRicoultSJHAsaraJMManningBD. mTORC1 induces purine synthesis through control of the mitochondrial tetrahydrofolate cycle. Science (2016) 351:728–33.10.1126/science.aad0489

  • 30

    Ben-SahraIHowellJJAsaraJMManningBD. Stimulation of de novo pyrimidine synthesis by growth signaling through mTOR and S6K1. Science (2013) 339:1323–8.10.1126/science.1228792

  • 31

    GuertinDASabatiniDM. Defining the role of mTOR in cancer. Cancer Cell (2007) 12(1):9–22.10.1016/j.ccr.2007.05.008

  • 32

    ArdestaniALupseBKidoYLeibowitzGMaedlerK. mTORC1 signaling: a double-edged sword in diabetic β cells. Cell Metab (2018) 27:314–31.10.1016/j.cmet.2017.11.004

  • 33

    KhamzinaLVeilleuxABergeronSMaretteA. Increased activation of the mammalian target of rapamycin pathway in liver and skeletal muscle of obese rats: possible involvement in obesity-linked insulin resistance. Endocrinology (2005) 146:1473–81.10.1210/en.2004-0921

  • 34

    DangCV. MYC on the path to cancer. Cell (2012) 149(1):22–35.10.1016/j.cell.2012.03.003

  • 35

    LiuYCLiFHandlerJHuangCRXiangYNerettiNet alGlobal regulation of nucleotide biosynthetic genes by c-Myc. PLoS One (2008) 3(7):e2722.10.1371/journal.pone.0002722

  • 36

    RiuEFerreTHidalgoAMasAFranckhauserSOtaeguiPet alOverexpression of c-myc in the liver prevents obesity and insulin resistance. FASEB J (2003) 17:1715–7.10.1096/fj.02-1163fje

  • 37

    JiangPDuWWangXMancusoAGaoXWuMet alP53 regulates biosynthesis through direct inactivation of glucose-6-phosphate dehydrogenase. Nat Cell Biol (2011) 13:310–6.10.1038/ncb2172

  • 38

    KollareddyMDimitrovaEVallabhaneniKCChanALeTChauhanKMet alRegulation of nucleotide metabolism by mutant p53 contributes to its gain-of-function activities. Nat Commun (2015) 6:7389.10.1038/ncomms8389

  • 39

    LiuJZhangCFengZ. Tumor suppressor p53 and its gain-of-function mutants in cancer. Acta Biochim Biophys Sin (Shanghai) (2014) 46(3):170–9.10.1093/abbs/gmt144

  • 40

    WangP-YMaWParkJ-YCeliFSArenaRChoiJWet alIncreased oxidative metabolism in the Li-Fraumeni syndrome. N Engl J Med (2013) 368:1027–32.10.1056/NEJMoa1214091

  • 41

    TongXZhaoFThompsonCB. The molecular determinants of de novo nucleotide biosynthesis in cancer cells. Curr Opin Genet Dev (2009) 19(1):32–7.10.1016/j.gde.2009.01.002

  • 42

    MackenzieRWElliottBT. Akt/PKB activation and insulin signaling: a novel insulin signaling pathway in the treatment of type 2 diabetes. Diabetes Metab Syndr Obes (2014) 7:55–64.10.2147/DMSO.S48260

  • 43

    MatsudaSKobayashiMKitagishiY. Roles for PI3K/AKT/PTEN pathway in cell signaling of nonalcoholic fatty liver disease. ISRN Endocrinol (2013) 2013:472432.10.1155/2013/472432

  • 44

    IzumiyaYHopkinsTMorrisCSatoKZengLViereckJet alFast/glycolytic muscle fiber growth reduces fat mass and improves metabolic parameters in obese mice. Cell Metab (2008) 7:159–72.10.1016/J.CMET.2007.11.003

  • 45

    GravesLMGuyHIKozlowskiPHuangMLazarowskiEPopeRMet alRegulation of carbamoyl phosphate synthetase by MAP kinase. Nature (2000) 403:328–32.10.1038/35002111

  • 46

    BurottoMChiouVLLeeJ-MKohnEC. The MAPK pathway across different malignancies: a new perspective. Cancer (2014) 120(22):3446–56.10.1002/cncr.28864

  • 47

    CarlsonCJKoterskiSSciottiRJPoccardGBRondinoneCM. Enhanced basal activation of mitogen-activated protein kinases in adipocytes from type 2 diabetes: potential role of p38 in the downregulation of GLUT4 expression. Diabetes (2003) 52:634–41.10.2337/diabetes.52.3.634

  • 48

    BostFAouadiMCaronLBinétruyB. The role of MAPKs in adipocyte differentiation and obesity. Biochimie (2005) 87:51–6.10.1016/J.BIOCHI.2004.10.018

  • 49

    TianWNBraunsteinLDPangJStuhlmeierKMXiQCTianXet alImportance of glucose-6-phosphate dehydrogenase activity for cell growth. J Biol Chem (1998) 273:10609–17.10.1074/JBC.273.17.10609

  • 50

    PatraKCHayN. The pentose phosphate pathway and cancer. Trends Biochem Sci (2014) 39:347–54.10.1016/j.tibs.2014.06.005

  • 51

    ParkJRhoHKKimKHChoeSSLeeYSKimJB. Overexpression of glucose-6-phosphate dehydrogenase is associated with lipid dysregulation and insulin resistance in obesity. Mol Cell Biol (2005) 25:5146–57.10.1128/MCB.25.12.5146-5157.2005

  • 52

    CaretteCDubois-LaforgueDGautierJ-FTimsitJ. Diabetes mellitus and glucose-6-phosphate dehydrogenase deficiency: from one crisis to another. Diabetes Metab (2011) 37:79–82.10.1016/j.diabet.2010.09.004

  • 53

    AirdKMZhangR. Nucleotide metabolism, oncogene-induced senescence and cancer. Cancer Lett (2015) 356:204–10.10.1016/j.canlet.2014.01.017

  • 54

    EngströmYErikssonSJildevikISkogSThelanderLTribukaitB. Cell cycle-dependent expression of mammalian ribonucleotide reductase. Differential regulation of the two subunits. J Biol Chem (1985) 260:9114–6.

  • 55

    KolbergMStrandKRGraffPAnderssonKK. Structure, function, and mechanism of ribonucleotide reductases. Biochim Biophys Acta Proteins Proteomics (2004) 1699(1–2):1–34.10.1016/j.bbapap.2004.02.007

  • 56

    TanakaHArakawaHYamaguchiTShiraishiKFukudaSMatsuiKet alA ribonucleotide reductase gene involved in a p53-dependent cell-cycle checkpoint for DNA damage. Nature (2000) 404:42–9.10.1038/35003506

  • 57

    BourdonAMinaiLSerreVJaisJPSarziEAubertSet alMutation of RRM2B, encoding p53-controlled ribonucleotide reductase (p53R2), causes severe mitochondrial DNA depletion. Nat Genet (2007) 39:776–80.10.1038/ng2040

  • 58

    HåkanssonPHoferAThelanderL. Regulation of mammalian ribonucleotide reduction and dNTP pools after DNA damage and in resting cells. J Biol Chem (2006) 281:7834–41.10.1074/jbc.M512894200

  • 59

    PontarinGFerraroPBeeLReichardPBianchiV. Mammalian ribonucleotide reductase subunit p53R2 is required for mitochondrial DNA replication and DNA repair in quiescent cells. Proc Natl Acad Sci U S A (2012) 109:13302–7.10.1073/pnas.1211289109

  • 60

    BornsteinBAreaEFlaniganKMGaneshJJayakarPSwobodaKJet alMitochondrial DNA depletion syndrome due to mutations in the RRM2B gene. Neuromuscul Disord (2008) 18:453–9.10.1016/j.nmd.2008.04.006

  • 61

    PalmieriFMonneM. Discoveries, metabolic roles and diseases of mitochondrial carriers: a review. Biochim Biophys Acta (2016) 1863:2362–78.10.1016/j.bbamcr.2016.03.007

  • 62

    Gutierrez-AguilarMBainesCP. Physiological and pathological roles of mitochondrial SLC25 carriers. Biochem J (2013) 454:371–86.10.1042/BJ20121753

  • 63

    ClemenconBBabotMTrezeguetV. The mitochondrial ADP/ATP carrier (SLC25 family): pathological implications of its dysfunction. Mol Aspects Med (2013) 34:485–93.10.1016/j.mam.2012.05.006

  • 64

    BaldwinSABealPRYaoSYMKingAECassCEYoungJD. The equilibrative nucleoside transporter family, SLC29. Pflugers Arch (2004) 447:735–43.10.1007/s00424-003-1103-2

  • 65

    GrayJHOwenRPGiacominiKM. The concentrative nucleoside transporter family, SLC28. Pflugers Arch (2004) 447:728–34.10.1007/s00424-003-1107-y

  • 66

    Molho-PessachVLererIAbeliovichDAghaZAbu LibdehABroshtilovaVet alThe H syndrome is caused by mutations in the nucleoside transporter hENT3. Am J Hum Genet (2008) 83:529–34.10.1016/j.ajhg.2008.09.013

  • 67

    ArnerESErikssonS. Mammalian deoxyribonucleoside kinases. Pharmacol Ther (1995) 67:155–86.10.1016/0163-7258(95)00015-9

  • 68

    El-HattabAWCraigenWJScagliaF. Mitochondrial DNA maintenance defects. Biochim Biophys Acta (2017) 1863:1539–55.10.1016/j.bbadis.2017.02.017

  • 69

    FreisingerPFüttererNLankesEGempelKBergerTMSpalingerJet alHepatocerebral mitochondrial DNA depletion syndrome caused by deoxyguanosine kinase (DGUOK) mutations. Arch Neurol (2006) 63:1129–34.10.1001/archneur.63.8.1129

  • 70

    RonchiDGaroneCBordoniAGutierrez RiosPCalvoSERipoloneMet alNext-generation sequencing reveals DGUOK mutations in adult patients with mitochondrial DNA multiple deletions. Brain (2012) 135:3404–15.10.1093/brain/aws258

  • 71

    SpelbrinkJNLiFYTirantiVNikaliKYuanQPTariqMet alHuman mitochondrial DNA deletions associated with mutations in the gene encoding twinkle, a phage T7 gene 4-like protein localized in mitochondria. Nat Genet (2001) 28:223–31.10.1038/90058

  • 72

    CopelandWC. Inherited mitochondrial diseases of DNA replication. Annu Rev Med (2008) 59:131–46.10.1146/annurev.med.59.053006.104646

  • 73

    TyynismaaHMjosundKPWanrooijSLappalainenIYlikallioEJalankoAet alMutant mitochondrial helicase twinkle causes multiple mtDNA deletions and a late-onset mitochondrial disease in mice. Proc Natl Acad Sci U S A (2005) 102:17687–92.10.1073/pnas.0505551102

  • 74

    KrasichRCopelandWC. DNA polymerases in the mitochondria: a critical review of the evidence. Front Biosci (Landmark Ed) (2017) 22:692–709.10.2741/4510

  • 75

    SinghBOwensKMBajpaiPDesoukiMMSrinivasasainagendraVTiwariHKet alMitochondrial DNA polymerase POLG1 disease mutations and germline variants promote tumorigenic properties. PLoS One (2015) 10:e0139846.10.1371/journal.pone.0139846

  • 76

    SinghKKAyyasamyVOwensKMKoulMSVujcicM. Mutations in mitochondrial DNA polymerase-gamma promote breast tumorigenesis. J Hum Genet (2009) 54:516–24.10.1038/jhg.2009.71

  • 77

    TervasmakiAMantereTHartikainenJMKauppilaSLeeH-MKoivuluomaSet alRare missense mutations in RECQL and POLG associate with inherited predisposition to breast cancer. Int J Cancer (2018).10.1002/ijc.31259

  • 78

    LinkowskaKJawienAMarszalekAMalyarchukBATonskaKBartnikEet alMitochondrial DNA polymerase gamma mutations and their implications in mtDNA alterations in colorectal cancer. Ann Hum Genet (2015) 79(5):320–8.10.1111/ahg.12111

  • 79

    LongXWangXChenYGuoXZhouFFanYet alPolymorphisms in POLG were associated with the prognosis and mtDNA content in hepatocellular carcinoma patients. Bull Cancer (2017) 104:500–7.10.1016/j.bulcan.2017.02.005

  • 80

    AirdKMWorthAJSnyderNWLeeJVSivanandSLiuQet alATM couples replication stress and metabolic reprogramming during cellular senescence. Cell Rep (2015) 11:893–901.10.1016/j.celrep.2015.04.014

  • 81

    ChoiMKippsTKurzrockR. ATM mutations in cancer: therapeutic implications. Mol Cancer Ther (2016) 15:1781–91.10.1158/1535-7163.MCT-15-0945

  • 82

    GuleriaAChandnaS. ATM kinase: much more than a DNA damage responsive protein. DNA Repair (Amst) (2016) 39:1–20.10.1016/j.dnarep.2015.12.009

  • 83

    ParksDAGrangerDN. Xanthine oxidase: biochemistry, distribution and physiology. Acta Physiol Scand Suppl (1986) 548:87–99.

  • 84

    BattelliMGPolitoLBortolottiMBolognesiA. Xanthine oxidoreductase in cancer: more than a differentiation marker. Cancer Med (2016) 5:546–57.10.1002/cam4.601

  • 85

    ChenCLuJ-MYaoQ. Hyperuricemia-related diseases and xanthine oxidoreductase (XOR) inhibitors: an overview. Med Sci Monit (2016) 22:2501–12.10.12659/MSM.899852

  • 86

    KaukonenJJuseliusJKTirantiVKyttalaAZevianiMComiGPet alRole of adenine nucleotide translocator 1 in mtDNA maintenance. Science (2000) 289:782–5.10.1126/science.289.5480.782

  • 87

    FavreCZhdanovALeahyMPapkovskyDO’ConnorR. Mitochondrial pyrimidine nucleotide carrier (PNC1) regulates mitochondrial biogenesis and the invasive phenotype of cancer cells. Oncogene (2010) 29:3964–76.10.1038/onc.2010.146

  • 88

    FloydSFavreCLasorsaFMLeahyMTrigianteGStroebelPet alThe insulin-like growth factor-I-mTOR signaling pathway induces the mitochondrial pyrimidine nucleotide carrier to promote cell growth. Mol Biol Cell (2007) 18:3545–55.10.1091/mbc.E06-12-1109

  • 89

    KenersonHLSubramanianSMcIntyreRKazamiMYeungRS. Livers with constitutive mTORC1 activity resist steatosis independent of feedback suppression of akt. PLoS One (2015) 10(2):e0117000.10.1371/journal.pone.0117000

  • 90

    UmSHFrigerioFWatanabeMPicardFJoaquinMStickerMet alAbsence of S6K1 protects against age- and diet-induced obesity while enhancing insulin sensitivity. Nature (2004) 431:200–5.10.1038/nature02866

  • 91

    KimLCCookRSChenJ. MTORC1 and mTORC2 in cancer and the tumor microenvironment. Oncogene (2017) 36(16):2191–201.10.1038/onc.2016.363

  • 92

    MillisSZIkedaSReddySGatalicaZKurzrockR. Landscape of phosphatidylinositol-3-kinase pathway alterations across 19 784 diverse solid tumors. JAMA Oncol (2016) 2:1565–73.10.1001/jamaoncol.2016.0891

  • 93

    MalleyCOPidgeonGP. The mTOR pathway in obesity driven gastrointestinal cancers: potential targets and clinical trials. BBA Clin (2016) 5:29–40.10.1016/j.bbacli.2015.11.003

  • 94

    SearsRC. The life cycle of c-Myc: from synthesis to degradation. Cell Cycle (2004) 3(9):1133–7.10.4161/cc.3.9.1145

  • 95

    MillerDMThomasSDIslamAMuenchDSedorisK. c-Myc and cancer metabolism. Clin Cancer Res (2012).10.1158/1078-0432.CCR-12-0977

  • 96

    WiseDRDeBerardinisRJMancusoASayedNZhangX-YPfeifferHKet alMyc regulates a transcriptional program that stimulates mitochondrial glutaminolysis and leads to glutamine addiction. Proc Natl Acad Sci U S A (2008) 105:18782–7.10.1073/pnas.0810199105

  • 97

    RiuEFerreTMasAHidalgoAFranckhauserSBoschF. Overexpression of c-myc in diabetic mice restores altered expression of the transcription factor genes that regulate liver metabolism. Biochem J (2002) 368:931–7.10.1042/BJ20020605

  • 98

    YangLVennetiSNagrathD. Glutaminolysis: a hallmark of cancer metabolism. Annu Rev Biomed Eng (2017) 19:163–94.10.1146/annurev-bioeng-071516-044546

  • 99

    DeBerardinisRJMancusoADaikhinENissimIYudkoffMWehrliSet alBeyond aerobic glycolysis: transformed cells can engage in glutamine metabolism that exceeds the requirement for protein and nucleotide synthesis. Proc Natl Acad Sci U S A (2007) 104:19345–50.10.1073/pnas.0709747104

  • 100

    ChabesAGeorgievaBDomkinVZhaoXRothsteinRThelanderL. Survival of DNA damage in yeast directly depends on increased dNTP levels allowed by relaxed feedback inhibition of ribonucleotide reductase. Cell (2003) 112:391–401.10.1016/S0092-8674(03)00075-8

  • 101

    LeTMPoddarSCapriJRAbtERKimWWeiLet alATR inhibition facilitates targeting of leukemia dependence on convergent nucleotide biosynthetic pathways. Nat Commun (2017) 8:241.10.1038/s41467-017-00221-3

  • 102

    ShimizuIYoshidaYKatsunoTTatenoKOkadaSMoriyaJet alP53-induced adipose tissue inflammation is critically involved in the development of insulin resistance in heart failure. Cell Metab (2012) 15:51–64.10.1016/j.cmet.2011.12.006

  • 103

    JiangPDuWWuM. Regulation of the pentose phosphate pathway in cancer. Protein Cell (2014) 8(8):592–602.10.1007/s13238-014-0082-8

  • 104

    LeeJ-WChoiAHHamMKimJ-WChoeSSParkJet alG6PD up-regulation promotes pancreatic beta-cell dysfunction. Endocrinology (2011) 152(3):793–803.10.1210/en.2010-0606

  • 105

    HamMChoeSSShinKCChoiGKimJWNohJRet alGlucose-6-phosphate dehydrogenase deficiency improves insulin resistance with reduced adipose tissue inflammation in obesity. Diabetes (2016) 65:2624–38.10.2337/db16-0060

  • 106

    WangLPiZLiuSLiuZSongF. Targeted metabolome profiling by dual-probe microdialysis sampling and treatment using Gardenia jasminoides for rats with type 2 diabetes. Sci Rep (2017) 7:10105.10.1038/s41598-017-10172-w

  • 107

    XiaJ-FLiangQ-LLiangX-PWangY-MHuPLiPet alUltraviolet and tandem mass spectrometry for simultaneous quantification of 21 pivotal metabolites in plasma from patients with diabetic nephropathy. J Chromatogr B Analyt Technol Biomed Life Sci (2009) 877:1930–6.10.1016/j.jchromb.2009.05.047

  • 108

    LiLLiuDWYanHYWangZYZhaoSHWangB. Obesity is an independent risk factor for non-alcoholic fatty liver disease: evidence from a meta-analysis of 21 cohort studies. Obes Rev (2016) 17:510–9.10.1111/obr.12407

  • 109

    SunBKarinM. Obesity, inflammation, and liver cancer. J Hepatol (2012) 56:704–13.10.1016/j.jhep.2011.09.020

  • 110

    GallagherEJLeRoithD. Obesity and diabetes: the increased risk of cancer and cancer-related mortality. Physiol Rev (2015) 95:727–48.10.1152/physrev.00030.2014

  • 111

    DannSGSelvarajAThomasG. mTOR complex1-S6K1 signaling: at the crossroads of obesity, diabetes and cancer. Trends Mol Med (2007) 13(6):252–9.10.1016/j.molmed.2007.04.002

  • 112

    AirdKMZhangGLiHTuZBitlerBGGaripovAet alSuppression of nucleotide metabolism underlies the establishment and maintenance of oncogene-induced senescence. Cell Rep (2013) 3:1252–65.10.1016/j.celrep.2013.03.004

  • 113

    BesterACRonigerMOrenYSImMMSarniDChaoatMet alNucleotide deficiency promotes genomic instability in early stages of cancer development. Cell (2011) 145:435–46.10.1016/j.cell.2011.03.044

  • 114

    FatkhutdinovNSproesserKKreplerCLiuQBraffordPAHerlynMet alTargeting RRM2 and mutant BRAF is a novel combinatorial strategy for melanoma. Mol Cancer Res (2016) 14:767–75.10.1158/1541-7786.mcr-16-0099

  • 115

    XuXPageJLSurteesJALiuHLagedrostSLuYet alBroad overexpression of ribonucleotide reductase genes in mice specifically induces lung neoplasms. Cancer Res (2008) 68:2652–60.10.1158/0008-5472.CAN-07-5873

  • 116

    HoferACronaMLoganDTSjöbergBM. DNA building blocks: keeping control of manufacture. Crit Rev Biochem Mol Biol (2012) 47(1):50–63.10.3109/10409238.2011.630372

  • 117

    NordlundPReichardP. Ribonucleotide reductases. Annu Rev Biochem (2006) 75:681–706.10.1146/annurev.biochem.75.103004.142443

  • 118

    ErikssonSGraslundASkogSThelanderLTribukaitB. Cell cycle-dependent regulation of mammalian ribonucleotide reductase. The S phase-correlated increase in subunit M2 is regulated by de novo protein synthesis. J Biol Chem (1984) 259:11695–700.

  • 119

    D’AngiolellaVDonatoVForresterFMJeongYTPellacaniCKudoYet alCyclin F-mediated degradation of ribonucleotide reductase M2 controls genome integrity and DNA repair. Cell (2012) 149:1023–34.10.1016/j.cell.2012.03.043

  • 120

    FoskolouIPJorgensenCLeszczynskaKBOlcinaMMTarhonskayaHHaismaBet alRibonucleotide reductase requires subunit switching in hypoxia to maintain DNA replication. Mol Cell (2017) 66:206–220.e9.10.1016/j.molcel.2017.03.005

  • 121

    KimuraTTakedaSSagiyaYGotohMNakamuraYArakawaH. Impaired function of p53R2 in Rrm2b-null mice causes severe renal failure through attenuation of dNTP pools. Nat Genet (2003) 34:440–5.10.1038/ng1212

  • 122

    LiuXXueLYenY. Redox property of ribonucleotide reductase small subunit M2 and p53R2. Methods Mol Biol (2008) 477:195–206.10.1007/978-1-60327-517-0_15

  • 123

    ElledgeSJZhouZAllenJBNavasTA. DNA damage and cell cycle regulation of ribonucleotide reductase. Bioessays (1993) 15:333–9.10.1002/bies.950150507

  • 124

    AirdKMLiHXinFKonstantinopoulosPAZhangRG. Identification of ribonucleotide reductase M2 as a potential target for pro-senescence therapy in epithelial ovarian cancer. Cell Cycle (2014) 13:199–207.10.4161/cc.26953

  • 125

    DressmanHKHansCBildAOlsonJARosenEMarcomPKet alGene expression profiles of multiple breast cancer phenotypes and response to neoadjuvant chemotherapy. Clin Cancer Res (2006) 12:819–26.10.1158/1078-0432.CCR-05-1447

  • 126

    FujitaHOhuchidaKMizumotoKItabaSItoTNakataKet alGene expression levels as predictive markers of outcome in pancreatic cancer after gemcitabine-based adjuvant chemotherapy. Neoplasia (2010) 12:807–17.10.1593/neo.10458

  • 127

    LiuXZhouBXueLYenFChuPUnFet alRibonucleotide reductase subunits M2 and p53R2 are potential biomarkers for metastasis of colon cancer. Clin Color Cancer (2007) 6:374–81.10.3816/CCC.2007.n.007

  • 128

    SaganL. On the origin of mitosing cells. J Theor Biol (1967) 14:225–IN6.10.1016/0022-5193(67)90079-3

  • 129

    GalluzziLKeppOTrojel-HansenCKroemerG. Mitochondrial control of cellular life, stress, and death. Circ Res (2012) 111(9):1198–207.10.1161/CIRCRESAHA.112.268946

  • 130

    GandhiVVSamuelsDC. Correlated tissue expression of genes of cytoplasmic and mitochondrial nucleotide metabolisms in normal tissues is disrupted in transformed tissues. Nucleosides Nucleotides Nucleic Acids (2012) 31:112–29.10.1080/15257770.2011.644101

  • 131

    HiranoMDiMauroS. Metabolic myopathies. Adv Neurol (2002) 88:217–34.

  • 132

    LeeWJohnsonJGoughDJDonoghueJCagnoneGLMVaghjianiVet alMitochondrial DNA copy number is regulated by DNA methylation and demethylation of POLGA in stem and cancer cells and their differentiated progeny. Cell Death Dis (2015) 6:e1664.10.1038/cddis.2015.34

  • 133

    ZhouXKannistoKCurboSvon DobelnUHultenbyKIsetunSet alThymidine kinase 2 deficiency-induced mtDNA depletion in mouse liver leads to defect beta-oxidation. PLoS One (2013) 8:e58843.10.1371/journal.pone.0058843

  • 134

    BaoXROngSGoldbergerOPengJSharmaRThompsonDAet alMitochondrial dysfunction remodels one-carbon metabolism in human cells. Elife (2016) 5:e10575.10.7554/eLife.10575

  • 135

    NikkanenJForsströmSEuroLPaetauIKohnzRAWangLet alMitochondrial DNA replication defects disturb cellular dNTP pools and remodel one-carbon metabolism. Cell Metab (2016) 23:635–48.10.1016/j.cmet.2016.01.019

  • 136

    WallaceDC. Mitochondrial diseases in man and mouse. Science (1999) 283(5407):1482–8.10.1126/science.283.5407.1482

  • 137

    MontgomeryMKTurnerN. Mitochondrial dysfunction and insulin resistance: an update. Endocr Connect (2014) 4:R1–15.10.1530/EC-14-0092

  • 138

    KoliakiCRodenM. Alterations of mitochondrial function and insulin sensitivity in human obesity and diabetes mellitus. Annu Rev Nutr (2016):1–31.10.1146/annurev-nutr-071715-050656

  • 139

    KamiharaJRanaHQGarberJE. Germline TP53 mutations and the changing landscape of Li-Fraumeni syndrome. Hum Mutat (2014) 35(6):654–62.10.1002/humu.22559

  • 140

    MalkinD. Li-Fraumeni syndrome. Genes Cancer (2011) 2:475–84.10.1177/1947601911413466

  • 141

    DahlESAirdKM. Ataxia-telangiectasia mutated modulation of carbon metabolism in cancer. Front Oncol (2017) 7:291.10.3389/fonc.2017.00291

  • 142

    BarRSLevisWRRechlerMMHarrisonLCSiebertCPodskalnyJet alExtreme insulin resistance in ataxia telangiectasia: defect in affinity of insulin receptors. N Engl J Med (1978) 298:1164–71.10.1056/NEJM197805252982103

  • 143

    McKinnonPJ. ATM and the molecular pathogenesis of ataxia telangiectasia. Annu Rev Pathol (2012) 7:303–21.10.1146/annurev-pathol-011811-132509

  • 144

    McKinnonPJ. ATM and ataxia telangiectasia. EMBO Rep (2004) 5:772–6.10.1038/sj.embor.7400210

  • 145

    DoreMPDavoliALongoNMarrasGPesGM. Glucose-6-phosphate dehydrogenase deficiency and risk of colorectal cancer in Northern Sardinia: a retrospective observational study. Medicine (Baltimore) (2016) 95:e5254.10.1097/MD.0000000000005254

  • 146

    ManganelliGMasulloUPassarelliSFilosaS. Glucose-6-phosphate dehydrogenase deficiency: disadvantages and possible benefits. Cardiovasc Hematol Disord Drug Targets (2013) 13:73–82.10.2174/1871529X11313010008

  • 147

    PesGMBassottiGDoreMP. Colorectal cancer mortality in relation to glucose-6-phosphate dehydrogenase deficiency and consanguinity in Sardinia: a spatial correlation analysis. Asian Pac J Cancer Prev (2017) 18:2403–7.10.22034/APJCP.2017.18.9.2403

  • 148

    FiniMAEliasAJohnsonRJWrightRM. Contribution of uric acid to cancer risk, recurrence, and mortality. Clin Transl Med (2012) 1:16.10.1186/2001-1326-1-16

  • 149

    SautinYYJohnsonRJ. Uric acid: the oxidant-antioxidant paradox. Nucleosides Nucleotides Nucleic Acids (2008) 27(6):608–19.10.1080/15257770802138558

  • 150

    ValkoMRhodesCJMoncolJIzakovicMMazurM. Free radicals, metals and antioxidants in oxidative stress-induced cancer. Chem Biol Interact (2006) 160(1):1–40.10.1016/j.cbi.2005.12.009

  • 151

    LuWXuYShaoXGaoFLiYHuJet alUric acid produces an inflammatory response through activation of NF-κB in the hypothalamus: implications for the pathogenesis of metabolic disorders. Sci Rep (2015) 5.10.1038/srep12144

  • 152

    ReuterSGuptaSCChaturvediMMAggarwalBB. Oxidative stress, inflammation, and cancer: how are they linked?Free Radic Biol Med (2010) 49(11):1603–16.10.1016/j.freeradbiomed.2010.09.006

  • 153

    GalmariniCMMackeyJRDumontetC. Nucleoside analogues and nucleobases in cancer treatment. Lancet Oncol (2002) 3(7):415–24.10.1016/S1470-2045(02)00788-X

  • 154

    Muñoz-PinedoCEl MjiyadNRicciJ-E. Cancer metabolism: current perspectives and future directions. Cell Death Dis (2012) 3:e248.10.1038/cddis.2011.123

  • 155

    de Sousa CavalcanteLMonteiroG. Gemcitabine: metabolism and molecular mechanisms of action, sensitivity and chemoresistance in pancreatic cancer. Eur J Pharmacol (2014) 741:8–16.10.1016/j.ejphar.2014.07.041

  • 156

    AmrutkarMGladhaugIP. Pancreatic cancer chemoresistance to gemcitabine. Cancers (Basel) (2017) 9.10.3390/cancers9110157

  • 157

    DeBerardinisRJChandelNS. Fundamentals of cancer metabolism. Sci Adv (2016) 2(5):e1600200.10.1126/sciadv.1600200

Summary

Keywords

purines, pyrimidines, c-Myc, p53, mTORC1, diabetes, obesity

Citation

Buj R and Aird KM (2018) Deoxyribonucleotide Triphosphate Metabolism in Cancer and Metabolic Disease. Front. Endocrinol. 9:177. doi: 10.3389/fendo.2018.00177

Received

09 February 2018

Accepted

03 April 2018

Published

18 April 2018

Volume

9 - 2018

Edited by

Che-Pei Kung, Washington University in St. Louis, United States

Reviewed by

Marco Falasca, Curtin University, Australia; Eva Surmacz, Temple University, United States

Updates

Copyright

*Correspondence: Katherine M. Aird,

Specialty section: This article was submitted to Cancer Endocrinology, a section of the journal Frontiers in Endocrinology

Disclaimer

All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.

Outline

Figures

Cite article

Copy to clipboard


Export citation file


Share article

Article metrics