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Early detection of structural abnormalities and cytoplasmic accumulation of TDP-43 in tissue-engineered skins derived from ALS patients

Bastien Paré1, Lydia Lydia Touzel Deschênes1*, Rémy Lamontagne1*, Marie-Soleil Lamarre1*, François-Dominique Scott1*, Hélène T. Khuong2*, Patrick A. Dion3*, Jean-Pierre Bouchard2, Peter Gould4*, Guy A. Rouleau3*, Nicolas Dupré2*, François Berthod1* and François Gros-Louis1*
  • 1 Laval University, Department of Surgery, Canada
  • 2 Laval University, Department of Neurological Sciences, Canada
  • 3 McGill University, Department of Neurology and Neurosurgery, Canada
  • 4 CHU de Québec, Department of Anatomic Pathology and Neuropathology, Canada

Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset disease characterized by the selective degeneration of motor neurons (MNs) in the central nervous system (CNS). Current diagnosis of ALS is based on clinical assessment of related symptoms which appear late in the disease course after degeneration of a significant number of MNs. As a result, the identification and development of disease-modifying therapies is difficult. Novel strategies for early diagnosis of ALS, to monitor disease progression and to assess response to existing and future treatments, are urgently needed.

Due to the common embryonic origin of both skin and neural tissues, many neurological disorders, including ALS, are accompanied by skin changes that often precede the apparition of neurological symptoms. We have developed a unique tissue-engineered skin model (TES) derived from symptomatic, sporadic and familial ALS patients as well as pre-symptomatic FALS patients carrying a known pathological DNA mutation. TES were generated from isolated keratinocytes and fibroblasts and analyzed using different biochemical, immunohistological and molecular methods. Our ALS-TES presents a number of striking structural and molecular features, uniquely seen in patients-derived TES, including extracellular matrix (ECM) disorganization and cytoplasmic TDP-43 inclusions, a pathological signature found in the majority of ALS cases in the affected regions of the CNS.

Consequently, our ALS-TES could represent a renewable source of human tissue to better understand the physiopathological mechanisms underlying ALS, facilitate the identification of disease biomarkers for early diagnosis and disease monitoring, as well as provide a unique tool for the development of drug screening assays.

Keywords: Tissue Engineering, self-assembly, in vitro, Drug testing

Conference: 10th World Biomaterials Congress, Montréal, Canada, 17 May - 22 May, 2016.

Presentation Type: Poster

Topic: Biomaterials in constructing tissue substitutes

Citation: Paré B, Lydia Touzel Deschênes L, Lamontagne R, Lamarre M, Scott F, T. Khuong H, Dion PA, Bouchard J, Gould P, Rouleau GA, Dupré N, Berthod F and Gros-Louis F (2016). Early detection of structural abnormalities and cytoplasmic accumulation of TDP-43 in tissue-engineered skins derived from ALS patients. Front. Bioeng. Biotechnol. Conference Abstract: 10th World Biomaterials Congress. doi: 10.3389/conf.FBIOE.2016.01.01162

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Received: 27 Mar 2016; Published Online: 30 Mar 2016.

* Correspondence:
Dr. Lydia Lydia Touzel Deschênes, Laval University, Department of Surgery, Québec, QC, Canada, Email1
Dr. Rémy Lamontagne, Laval University, Department of Surgery, Québec, QC, Canada, Email2
Dr. Marie-Soleil Lamarre, Laval University, Department of Surgery, Québec, QC, Canada, Email3
Dr. François-Dominique Scott, Laval University, Department of Surgery, Québec, QC, Canada, Email4
Dr. Hélène T. Khuong, Laval University, Department of Neurological Sciences, Québec, QC, Canada, Email5
Dr. Patrick A Dion, McGill University, Department of Neurology and Neurosurgery, Montréal, QC, Canada, Email6
Dr. Peter Gould, CHU de Québec, Department of Anatomic Pathology and Neuropathology, Québec, QC, Canada, Email7
Dr. Guy A Rouleau, McGill University, Department of Neurology and Neurosurgery, Montréal, QC, Canada, Email8
Dr. Nicolas Dupré, Laval University, Department of Neurological Sciences, Québec, QC, Canada, Email9
Dr. François Berthod, Laval University, Department of Surgery, Québec, QC, Canada, Email10
Dr. François Gros-Louis, Laval University, Department of Surgery, Québec, QC, Canada, Email11