title: TDP-43 in central nervous system development and function: clues to TDP-43-associated neurodegeneration.
abstract: From the earliest stages of embryogenesis and throughout life, transcriptional regulation is carefully orchestrated in order to generate, shape, and reshape the central nervous system (CNS). TAR DNA-binding protein 43 (TDP-43) is identified as a regulator of essential transcriptional events in the CNS. Evidence for its importance comes from the identification of TDP-43 protein aggregates and genetic mutations in patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Efforts are being made to learn more about the biological function of TDP-43 and gain a better understanding of its role in neurodegeneration. TDP-43 RNA targets and protein interactions have now been identified, and in vivo evidence shows that TDP-43 is essential in CNS development and function. This review will highlight aspects of these findings.
authors.affiliation: Deparment of Neuroscience, The University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390-9111, USA. Chantelle.Sephton@UTSouthwestern.edu
authors.forename: Chantelle F
authors.initials: CF
authors.lastname: Sephton
authors.forename: Basar
authors.initials: B
authors.lastname: Cenik
authors.forename: Bercin Kutluk
authors.initials: BK
authors.lastname: Cenik
authors.forename: Joachim
authors.initials: J
authors.lastname: Herz
authors.forename: Gang
authors.initials: G
authors.lastname: Yu
mesh_terms: D000818:Animals; D001921:Brain / Q000254:growth & development / Q000378:metabolism / Q000502:physiology* / Q000503:physiopathology*; D004268:DNA-Binding Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D019636:Neurodegenerative Diseases / Q000378:metabolism* / Q000503:physiopathology; D012313:RNA / Q000378:metabolism; D012261:Ribonucleoproteins / Q000378:metabolism; D013116:Spinal Cord / Q000254:growth & development / Q000378:metabolism / Q000502:physiology* / Q000503:physiopathology*
chemical_list: D004268:DNA-Binding Proteins; D012261:Ribonucleoproteins; D012313:RNA