AUTHOR=Nakisli Sera , Lagares Alfonso , Nielsen Corinne M. , Cuervo Henar 

TITLE=Pericytes and vascular smooth muscle cells in central nervous system arteriovenous malformations

JOURNAL=Frontiers in Physiology

VOLUME=Volume 14 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/physiology/articles/10.3389/fphys.2023.1210563

DOI=10.3389/fphys.2023.1210563

ISSN=1664-042X

ABSTRACT=Previously considered passive support cells, mural cellspericytes and vascular smooth muscle cellshave started to garner more attention in disease research, as more subclassifications, based on morphology, gene expression, and function, have been discovered. Central nervous system (CNS) arteriovenous malformations (AVMs) represent a neurovascular disorder in which mural cells have been shown to be affected, both in animal models and in human patients. To study consequences to mural cells in the context of AVMs, various animal models have been developed to mimic and predict human AVM pathologies. A key takeaway from recently published work is that AVMs and mural cells are heterogeneous in their molecular, cellular, and functional characteristics. In this review, we summarize the observed perturbations to mural cells in human CNS AVM samples and CNS AVM animal models, and we discuss various potential mechanisms relating mural cell pathologies to AVMs.Arteriovenous malformations (AVMs) are vascular anomalies that are characterized by having a widened arteriovenous (AV) connection. This increased AV diameter permits increased blood flow and pressure, leading to microhemorrhages, poor nutrient-waste exchange, and tortuous vessels, which may form into a vessel entanglement called a nidus (Nielsen et al., 2016;Varshneya, 2019). Clinically, AVMs can develop in many tissues throughout the body, though most develop as focal lesions in patients. In fact, patients that present with multi-tissue AVMs usually have an inherited syndrome, such as Hereditary Hemorrhagic Telangiectasia (HHT), discussed in more detail below, in which AVMs can be observed in nose, lung, brain, and liver (Kim et al., 2018;Snodgrass et al., 2021). Consequences of AVMs depend on the severity of the