NP_082254.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
40,504 Da
NCBI Official Full Name
4-hydroxybenzoate polyprenyltransferase, mitochondrial
NCBI Official Synonym Full Names
coenzyme Q2 4-hydroxybenzoate polyprenyltransferase
NCBI Protein Information
4-hydroxybenzoate polyprenyltransferase, mitochondrial
UniProt Protein Name
4-hydroxybenzoate polyprenyltransferase, mitochondrial
UniProt Synonym Protein Names
Para-hydroxybenzoate--polyprenyltransferase
UniProt Synonym Gene Names
4-HB polyprenyltransferase; PHB:PPT; PHB:polyprenyltransferase [Similar Products]
UniProt Entry Name
COQ2_MOUSE
UniProt Comments for Coq2
COQ2: 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. Defects in COQ2 are the cause of coenzyme Q10 deficiency, primary, type 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. Belongs to the UbiA prenyltransferase family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Membrane protein, multi-pass; Mitochondrial; Transferase; EC 2.5.1.39; Membrane protein, integral; Cofactor and Vitamin Metabolism - ubiquinone and other terpenoid-quinone biosynthesis
Cellular Component: integral to membrane; membrane; mitochondrial inner membrane; mitochondrion
Molecular Function: 4-hydroxybenzoate decaprenyltransferase activity; 4-hydroxybenzoate nonaprenyltransferase activity; prenyltransferase activity; transferase activity
Biological Process: glycerol metabolic process; isoprenoid biosynthetic process; ubiquinone biosynthetic process
Research Articles on Coq2
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Pathways associated with Coq2 recombinant protein
Diseases associated with Coq2 recombinant protein
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