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

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

Research output: Contribution to journalReview articlepeer-review

52 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)589-594
Number of pages6
JournalBiological Chemistry
Issue number7
StatePublished - Jul 2012


  • Amyotrophic lateral sclerosis (ALS)
  • Neural development
  • PTPB2
  • RIP-seq
  • RNA binding protein
  • TDP-43

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Clinical Biochemistry


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