Introduction
Purine-rich element binding protein alpha (Purα or PurA) is a transcription factor with a molecular weight of ~35 kDa encoded by the PURA gene. It possesses ATP-independent dsDNA unwinding activity, and is known to bind sequence-specific purine-rich regions of ssDNA and ssRNA, recognizing GGN motifs. Purα is a member of the PUR family of proteins, which includes Purβ and two isoforms of Purγ. In its functional dimeric form Purα is known to repress expression of smooth muscle alpha actin (SMαA) encoded by the Acta2 gene. It is also known to be involved in DNA replication and cell cycle regulation as well as mRNA translation. It plays a crucial role in nervous system development, and mutations in Purα have been implicated in two neurological diseases: PURA syndrome and Fragile X-associated Tremor/Ataxia Syndrome (FXTAS) (see Disease section).
Structure
facilitates dimerization of Purα monomers. The association of two repeat III domains forms what is termed the "intermolecular domain".
A PUR domain is analogous to a left-handed handshake. PUR repeat I-II represented from 5fgp.
Function
functions as a homodimer or heterodimer with PurB (and PurG?), PurA is known to repress various genes including
, Y57 (repeat I) and F145 (repeat II) have been implicated in the DNA unwinding activity of PurA.[1]
Disease
Relevance
Structural highlights
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