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Protein basic information

LiverAtlas Protein ID

HuLPr16037

Uniprot ID

COQ2_HUMAN

Uniprot Acc

Q96H96;O95331;Q1JQ78;Q684R2;

Protein name

4-hydroxybenzoate polyprenyltransferase, mitochondrial

Comment

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.||CATALYTIC ACTIVITY:A polyprenyl diphosphate + 4-hydroxybenzoate = diphosphate + a 4-hydroxy-3-polyprenylbenzoate.||PATHWAY:Cofactor biosynthesis; ubiquinone biosynthesis.||SUBCELLULAR LOCATION:Mitochondrion membrane; Multi-pass membrane protein (Probable).||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;||TISSUE SPECIFICITY:Widely expressed. Present in all of the tissues tested. Expressed at higher level in skeletal muscle, adrenal glands and the heart.||DISEASE:Defects in COQ2 are a cause of coenzyme Q10 deficiency [MIM:607426]. Coenzyme Q10 deficiency is an autosomal recessive disorder with variable manife

Subcellular localization

Mitochondrion membrane;Multi-pass membrane protein(Probable).

Gene name

coenzyme Q2 homolog, prenyltransferase (yeast)

Protein sequence

MLGSRAAGFARGLRALALAWLPGWRGRSFALARAAGAPHG GDLQPPACPEPRGRQLSLSAAAVVDSAPRPLQPYLRLMR LDKPIGTWLLYLPCTWSIGLAAEPGCFPDWYMLSLFGTG AILMRGAGCTINDMWDQDYDKKVTRTANRPIAAGDISTF QSFVFLGGQLTLALGVLLCLNYYSIALGAGSLLLVITYP LMKRISYWPQLALGLTFNWGALLGWSAIKGSCDPSVCLP LYFSGVMWTLIYDTIYAHQDKRDDVLIGLKSTALRFGEN TKPWLSGFSVAMLGALSLVGVNSGQTAPYYAALGAVGAH LTHQIYTLDIHRPEDCWNKFISNRTLGLIVFLGIVLGNL WKEKKTDKTKKGIENKIEN

Database cross reference

RefSeq Protein accession:NP_056512
RefSeq Protein gi:260656052

Liver relevance

HLPP validation

Yes/No

Yes

Project name

Chinese Liver;Human Liver Organelles;

Ontology annotation

GO-C

GO:0031966;C:mitochondrial membrane;IEA:UniProtKB-SubCell.

GO-F

GO:0002083;F:4-hydroxybenzoate decaprenyltransferase activity;IEA:EC. GO:0047293;F:4-hydroxybenzoate nonaprenyltransferase activity;IEA:EC.

GO-P

GO:0006071;P:glycerol metabolic process;IGI:UniProtKB. GO:0008299;P:isoprenoid biosynthetic process;IEA:UniProtKB-KW. GO:0006744;P:ubiquinone biosynthetic process;IDA:UniProtKB.

Pathway

Pathway name

Ubiquinone_Biosynthesis