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LMPD Database

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LMP004370

UniProt Annotations

Entry Information

Gene Name	coenzyme Q2 4-hydroxybenzoate polyprenyltransferase
Protein Entry	COQ2_HUMAN
UniProt ID	Q96H96
Species	Human

Comments

Comment type	Description
Alternative Products	Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q96H96-1; Sequence=Displayed; Name=3; IsoId=Q96H96-3; Sequence=VSP_017677, VSP_017678;
Catalytic Activity	A polyprenyl diphosphate + 4-hydroxybenzoate = diphosphate + a 4-hydroxy-3-polyprenylbenzoate.
Disease	Coenzyme Q10 deficiency, primary, 1 (COQ10D1) [MIM
Disease	Multiple system atrophy 1 (MSA1) [MIM
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.
Pathway	Cofactor biosynthesis; ubiquinone biosynthesis.
Sequence Caution	Sequence=AAC72955.1; Type=Frameshift; Positions=172; Evidence= ; Sequence=AAH20728.2; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence= ; Sequence=CAF18241.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence= ;
Similarity	Belongs to the UbiA prenyltransferase family.
Subcellular Location	Mitochondrion membrane ; Multi- pass membrane protein .
Tissue Specificity	Widely expressed. Present in all of the tissues tested. Expressed at higher level in skeletal muscle, adrenal glands and the heart.