PARK7

Protein deglycase DJ-1, also known as Parkinson disease protein 7, is a protein which in humans is encoded by the PARK7 gene.

Gene

The gene PARK7, also known as DJ-1, encodes a protein of the peptidase C56 family. The human gene PARK7 has 8 Exons and locates at chromosome band 1p36.23.

Protein

The human protein deglycase DJ-1 is 20 kDa in size and composed of 189 amino acids with seven β-strands and nine α-helices in total and is present as a dimer. It belongs to the peptidase C56 family of proteins.

The protein structures of human protein DJ-1, Escherichia coli chaperone Hsp31 and an Archaea protease are evolutionarily conserved.

Function

Under an oxidative condition, protein deglycase DJ-1 inhibits the aggregation of α-synuclein via its chaperone activity, thus functions as a redox-sensitive chaperone and as a sensor for oxidative stress. Accordingly, DJ-1 apparently protects neurons against oxidative stress and cell death. In parallel, Protein DJ-1 acts as a positive regulator of androgen receptor-dependent transcription.

Pyrroloquinoline_quinone (PQQ) has been shown to reduce the self-oxidation of the DJ-1 protein, an early step in the onset of some forms of Parkinson's disease.

Clinical significance

Defects in this gene are the cause of autosomal recessive early-onset Parkinson's disease 7.

Interactions

PARK7 has been shown to interact with:

• CASK,
• EFCAB6, and
• PIAS2.

See also

• Parkinson's disease

Further reading

This article incorporates text from the United States National Library of Medicine, which is in the public domain.