Article

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

Deparment of Neuroscience, The University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390-9111, USA.
Biological Chemistry (Impact Factor: 2.69). 07/2012; 393(7):589-94. DOI: 10.1515/hsz-2012-0115
Source: PubMed

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.

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