Protein deglycase DJ-1, also known as Parkinson disease protein 7, is a
protein which in humans is encoded by the ''PARK7''
gene.
Structure
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-strand
The beta sheet, (β-sheet) (also β-pleated sheet) is a common motif of the regular protein secondary structure. Beta sheets consist of beta strands (β-strands) connected laterally by at least two or three backbone hydrogen bonds, forming a g ...
s and nine
α-helices in total and is present as a
dimer
Dimer may refer to:
* Dimer (chemistry), a chemical structure formed from two similar sub-units
** Protein dimer, a protein quaternary structure
** d-dimer
* Dimer model, an item in statistical mechanics, based on ''domino tiling''
* Julius Dimer ( ...
. It belongs to the
peptidase C56 family of proteins.
The protein structures of human protein DJ-1, ''
Escherichia coli'' chaperone Hsp31, YhbO and YajL 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 functioning 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
The androgen receptor (AR), also known as NR3C4 (nuclear receptor subfamily 3, group C, member 4), is a type of nuclear receptor that is activated by binding any of the androgenic hormones, including testosterone and dihydrotestosterone in th ...
-dependent transcription. DJ-1 is expressed in both the neural retina and retinal pigment epithelium of mammals, where it exerts a neuroprotective role against oxidative stress under both physiological and pathological conditions.
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.
Functional DJ-1 protein has been shown to bind metals and protect against metal-induced cytotoxicity from
copper and
mercury
Mercury commonly refers to:
* Mercury (planet), the nearest planet to the Sun
* Mercury (element), a metallic chemical element with the symbol Hg
* Mercury (mythology), a Roman god
Mercury or The Mercury may also refer to:
Companies
* Merc ...
.
[ ]
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
Advocates for Informed Choice, dba interACT or interACT Advocates for Intersex Youth, is a 501(c)(3) nonprofit organization using innovative strategies to advocate for the legal and human rights of children with intersex traits. The organizati ...
with:
*
CASK,
*
EFCAB6
EF-hand calcium-binding domain-containing protein 6 is a protein that in humans is encoded by the ''EFCAB6'' gene.
Interactions
EFCAB6 has been shown to interact with PARK7 and Androgen receptor
The androgen receptor (AR), also known as NR ...
,
and
*
PIAS2.
See also
*
Parkinson's disease
References
Further reading
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External links
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