TDP-43 in central nervous system development and function: clues to TDP-43-associated neurodegeneration.

Publication Type:

Journal Article

Authors:

Chantelle F Sephton; Cenik, Basar; Cenik, Bercin Kutluk; Herz, Joachim; Yu, Gang

Source:

Biol Chem, Volume 393, Issue 7, p.589-94 (2012)

Keywords:

Animals, Brain, DNA-Binding Proteins, Humans, Neurodegenerative Diseases, Ribonucleoproteins, RNA, Spinal Cord

Abstract:

<p>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.</p>