COQ2
CoQ (ubiquinone) serves as a redox carrier in the mitochondrial respiratory chain and is a lipid-soluble antioxidant. COQ2, or parahydroxybenzoate-polyprenyltransferase (EC 2.5.1.39), catalyzes one of the final reactions in the biosynthesis of CoQ, the prenylation of parahydroxybenzoate with an all-trans polyprenyl group (Forsgren et al., 2004 [PubMed 15153069]).[supplied by OMIM
Full Name
coenzyme Q2 homolog, prenyltransferase (yeast)
Function
Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB, generating the first membrane-bound Q intermediate.
Biological Process
Glycerol metabolic process Source: UniProtKB
Isoprenoid biosynthetic process Source: UniProtKB-UniRule
Ubiquinone biosynthetic process Source: UniProtKB
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Coenzyme Q10 deficiency, primary, 1 (COQ10D1):
An autosomal recessive disorder with variable manifestations consistent with 5 major phenotypes. The phenotypes include an encephalomyopathic form with seizures and ataxia; a multisystem infantile form with encephalopathy, cardiomyopathy and renal failure; a predominantly cerebellar form with ataxia and cerebellar atrophy; Leigh syndrome with growth retardation; and an isolated myopathic form.
Multiple system atrophy 1 (MSA1):
A progressive neurodegenerative disorder clinically characterized by parkinsonism, cerebellar ataxia, and autonomic, urogenital, and pyramidal dysfunction in various combinations. Pathologically, it is characterized by degeneration of striatonigral and olivopontocerebellar structures, and glial cytoplasmic inclusions that consist of abnormally phosphorylated alpha-synuclein or tau.
Topology
Mitochondrial matrix: 35-83
Helical: 84-104
Mitochondrial intermembrane: 105-108
Helical: 109-129
Mitochondrial matrix: 130-148
Helical: 149-169
Mitochondrial intermembrane: 170-172
Helical: 173-193
Mitochondrial matrix: 194-203
Helical: 204-224
Mitochondrial intermembrane: 225-231
Helical: 232-252
Mitochondrial matrix: 253-277
Helical: 278-298
Mitochondrial intermembrane: 299-300
Helical: 301-321
Mitochondrial matrix: 322-332
Helical: 333-353
Mitochondrial intermembrane: 354-371
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Datasheet