Gene name: COQ2

Uniprot entry:

Q96H96

Protein names:

4-hydroxybenzoate polyprenyltransferase, mitochondrial (4-HB polyprenyltransferase) (EC 2.5.1.39) (4-hydroxybenzoate decaprenyltransferase) (COQ2 homolog) (hCOQ2) (Para-hydroxybenzoate--polyprenyltransferase) (PHB:PPT) (PHB:polyprenyltransferase)

Protein sequence:

1_MLGSR 6_ AAGFA 11_ RGLRA 16_ LALAW 21_ LPGWR 26_ GRSFA 31_ LARAA 36_ GAPHG 41_ GDLQP 46_ PACPE 51_ PRGRQ 56_ LSLSA 61_ AAVVD 66_ SAPRP 71_ LQPYL 76_ RLMRL 81_ DKPIG 86_ TWLLY 91_ LPCTW 96_ SIGLA 101_ AEPGC 106_ FPDWY 111_ MLSLF 116_ GTGAI 121_ LMRGA 126_ GCTIN 131_ DMWDQ 136_ DYDKK 141_ VTRTA 146_ NRPIA 151_ AGDIS 156_ TFQSF 161_ VFLGG 166_ QLTLA 171_ LGVLL 176_ CLNYY 181_ SIALG 186_ AGSLL 191_ LVITY 196_ PLMKR 201_ ISYWP 206_ QLALG 211_ LTFNW 216_ GALLG 221_ WSAIK 226_ GSCDP 231_ SVCLP 236_ LYFSG 241_ VMWTL 246_ IYDTI 251_ YAHQD 256_ KRDDV 261_ LIGLK 266_ STALR 271_ FGENT 276_ KPWLS 281_ GFSVA 286_ MLGAL 291_ SLVGV 296_ NSGQT 301_ APYYA 306_ ALGAV 311_ GAHLT 316_ HQIYT 321_ LDIHR 326_ PEDCW 331_ NKFIS 336_ NRTLG 341_ LIVFL 346_ GIVLG 351_ NLWKE 356_ KKTDK 361_ TKKGI 366_ENKIE

Protein annotations

Protein functions:

1: Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis (PubMed:15153069, PubMed:16400613, PubMed:17374725, PubMed:20526342). Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl donor (such as all-trans-decaprenyl diphosphate) (PubMed:15153069, PubMed:16400613, PubMed:17374725, PubMed:20526342). The length of the polyprenyl side chain varies depending on the species, in humans, the side chain is comprised of 10 isoprenyls (decaprenyl) producing CoQ10 (also known as ubiquinone), whereas rodents predominantly generate CoQ9 (PubMed:15153069, PubMed:16400613). However, this specificity is not complete, human tissues have low amounts of CoQ9 and rodent organs contain some CoQ10 (PubMed:15153069). Plays a central role in the biosynthesis of CoQ10 (PubMed:15153069, PubMed:16400613, PubMed:17374725). CoQ10 is a vital molecule that transports electrons from mitochondrial respiratory chain complexes (PubMed:16400613, PubMed:17374725, PubMed:27493029). CoQs also function as cofactors for uncoupling protein and play a role as regulators of the extracellularly-induced ceramide-dependent apoptotic pathway (PubMed:16400613, PubMed:17374725). Regulates mitochondrial permeability transition pore (mPTP) opening and ROS production (pivotal events in cell death) in a tissue specific manner (By similarity)