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Biol Chem. 2012 Jul;393(7):589-94. doi: 10.1515/hsz-2012-0115.

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

Author information

1
Deparment of Neuroscience, The University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390-9111, USA. Chantelle.Sephton@UTSouthwestern.edu

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.

PMID:
22944662
PMCID:
PMC3537500
DOI:
10.1515/hsz-2012-0115
[Indexed for MEDLINE]
Free PMC Article
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