TDP-43: a DNA and RNA binding protein with roles in neurodegenerative diseases

Sadaf T. Warraich, Shu Yang, Garth A. Nicholson, Ian P. Blair*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

53 Citations (Scopus)


Transactive response DNA binding protein 43. kDa (TDP-43) is a DNA and RNA binding protein involved in RNA processing and with structural resemblance to heterogeneous ribonucleoproteins (hnRNPs). TDP-43 serves multiple functions with roles in transcriptional regulation, pre-mRNA splicing and translational regulation. TDP-43 is also crucial for embryonic development with increasing evidence indirectly implicating its involvement in other cellular processes including microRNA biogenesis, apoptosis and cell division. The role of TDP-43 in neurodegeneration has been actively studied since identification as a major component of the ubiquitinated inclusions seen in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). TDP-43 pathology has also been identified in several other neurodegenerative diseases. These disorders are collectively referred to as TDP-43 proteinopathies. The identification of rare TDP-43 mutations in sporadic and familial forms of ALS and FTLD suggests TDP-43 plays an important pathogenic role, rather than merely being a marker of the disease.

Original languageEnglish
Pages (from-to)1606-1609
Number of pages4
JournalInternational Journal of Biochemistry and Cell Biology
Issue number10
Publication statusPublished - Oct 2010
Externally publishedYes


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