Descriptions

COQ8A is an atypical kinase involved in the biosynthesis of coenzyme Q. Autoinhibition is achieved by the N-terminal region containing KxGQ motif, which occludes the substrate binding site of the kinase domain within the same polypeptide. Mutation of A339G in A-rich loop significantly enhances selectively for ATP, and thus Ala-339 is a major structural determinant of coenzyme selectivity.

Autoinhibitory domains (AIDs)

Target domain

394-648 (Activator of bc1 complex, ABC1, kinases, also called aarF domain containing kinase 3)

Relief mechanism

PTM

Assay

Accessory elements

No accessory elements

Autoinhibited structure

Activated structure

1 structures for Q18486

Entry ID Method Resolution Chain Position Source
AF-Q18486-F1 Predicted AlphaFoldDB

No variants for Q18486

Variant ID(s) Position Change Description Diseaes Association Provenance
No variants for Q18486

No associated diseases with Q18486

2 regional properties for Q18486

Type Name Position InterPro Accession
domain ABC1 atypical kinase-like domain 399 - 643 IPR004147
domain ADCK3-like domain 394 - 648 IPR034646

Functions

Description
EC Number 2.7.-.- Transferring phosphorus-containing groups
Subcellular Localization
  • Mitochondrion
PANTHER Family
PANTHER Subfamily
PANTHER Protein Class
PANTHER Pathway Category No pathway information available

1 GO annotations of cellular component

Name Definition
mitochondrion A semiautonomous, self replicating organelle that occurs in varying numbers, shapes, and sizes in the cytoplasm of virtually all eukaryotic cells. It is notably the site of tissue respiration.

2 GO annotations of molecular function

Name Definition
ATP binding Binding to ATP, adenosine 5'-triphosphate, a universally important coenzyme and enzyme regulator.
transferase activity Catalysis of the transfer of a group, e.g. a methyl group, glycosyl group, acyl group, phosphorus-containing, or other groups, from one compound (generally regarded as the donor) to another compound (generally regarded as the acceptor). Transferase is the systematic name for any enzyme of EC class 2.

1 GO annotations of biological process

Name Definition
ubiquinone biosynthetic process The chemical reactions and pathways resulting in the formation of ubiquinone, a lipid-soluble electron-transporting coenzyme.

11 homologous proteins in AiPD

UniProt AC Gene Name Protein Name Species Evidence Code
P27697 COQ8 Atypical kinase COQ8, mitochondrial Saccharomyces cerevisiae (strain ATCC 204508 / S288c) (Baker's yeast) SS
Q29RI0 COQ8A Atypical kinase COQ8A, mitochondrial Bos taurus (Bovine) SS
Q96D53 COQ8B Atypical kinase COQ8B, mitochondrial Homo sapiens (Human) SS
Q8NI60 COQ8A Atypical kinase COQ8A, mitochondrial Homo sapiens (Human) EV
Q60936 Coq8a Atypical kinase COQ8A, mitochondrial Mus musculus (Mouse) SS
Q566J8 Coq8b Atypical kinase COQ8B, mitochondrial Mus musculus (Mouse) SS
Q5BJQ0 Coq8a Atypical kinase COQ8A, mitochondrial Rattus norvegicus (Rat) SS
Q6AY19 Coq8b Atypical kinase COQ8B, mitochondrial Rattus norvegicus (Rat) SS
Q9SBB2 ABC1 Protein ABC transporter 1, mitochondrial Arabidopsis thaliana (Mouse-ear cress) SS
A3QJU3 coq8b Atypical kinase COQ8B, mitochondrial Danio rerio (Zebrafish) (Brachydanio rerio) SS
Q5RGU1 coq8a Atypical kinase COQ8A, mitochondrial Danio rerio (Zebrafish) (Brachydanio rerio) SS
10 20 30 40 50 60
MAAKRPPAST LKWCQQEMGP ICEGIGMVVR SQIGYDGRRI EKQIRRKALQ TVVMGGQDVD
70 80 90 100 110 120
PLKEPNKTNA PLLSPTLPST NEALQRARVV AAGIETFAKL MSQGTYPGSA GFTVSETGER
130 140 150 160 170 180
KYMKQTSPVQ SNIVANLMAT ADKLSKGVIS LAPFPLPTIT KLGTWVIANP TAQLPIPPQL
190 200 210 220 230 240
KSAIPAIGQI ETLIQSILGV QGPVSSVSRA PRKLKLEGVT DQVVLEPETI HATVDVEPID
250 260 270 280 290 300
KTLTKEEAEF LIKAARSVDD DNAETAIFGT QKAKPKVYRP ELPKNFEVNL EMGNVHTLTR
310 320 330 340 350 360
SNESSVPATR IGRLATFGQL AFGLLGGATA EVTRRTFGIG KRLQEEGIPK NPFLSEANAD
370 380 390 400 410 420
RIVATLCRVR GAALKLGQML SIQDSSTVPP ALLQIFERVR QSADFMPIKQ VHRQMKDAFG
430 440 450 460 470 480
DDWREKFEHF DDKPFACASI GQVHKAVLKD GRNVAVKVQY PGVAEGIDSD IDNLVSVLSV
490 500 510 520 530 540
GGIFPKGMFL DAFVGVARRE LKQECDYERE ARAMKKFREL IADWQDVYVP EVIDELSSSR
550 560 570 580 590 600
VLTTELVYGK PVDACVEEPQ VVRDYIAGKF IELCLKEIFL WRFMQTDPNW SNFFLGKHPK
610 620 630 640 650 660
TGEPRLVLLD FGASRAYGKK FVDIYMNIIK SAYDGDKKKI IEYSREIGFL TGYETSVMED
670 680 690 700 710 720
AHVESVMIMG ETLASNHPYN FANQDVTMRI QKLIPVMLEH RLTSPPEEIY SLHRKLSGCY
730 740 750
LLAAKLKATV SCGGLFHEIH ENYVFGEDGI DINID