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

268-518 (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 A3QJU3

Entry ID Method Resolution Chain Position Source
AF-A3QJU3-F1 Predicted AlphaFoldDB

No variants for A3QJU3

Variant ID(s) Position Change Description Diseaes Association Provenance
No variants for A3QJU3

No associated diseases with A3QJU3

14 regional properties for A3QJU3

Type Name Position InterPro Accession
domain Protein kinase domain 621 - 884 IPR000719
domain Ephrin receptor ligand binding domain 20 - 202 IPR001090
domain Serine-threonine/tyrosine-protein kinase, catalytic domain 622 - 880 IPR001245
conserved_site Tyrosine-protein kinase, receptor class V, conserved site 178 - 198 IPR001426-1
conserved_site Tyrosine-protein kinase, receptor class V, conserved site 241 - 261 IPR001426-2
domain Sterile alpha motif domain 910 - 977 IPR001660
domain Fibronectin type III 324 - 434 IPR003961-1
domain Fibronectin type III 435 - 530 IPR003961-2
active_site Tyrosine-protein kinase, active site 742 - 754 IPR008266
domain Tyrosine-protein kinase ephrin type A/B receptor-like 268 - 302 IPR011641
binding_site Protein kinase, ATP binding site 627 - 653 IPR017441
domain Tyrosine-protein kinase, catalytic domain 621 - 880 IPR020635
domain Ephrin receptor, transmembrane domain 544 - 617 IPR027936
domain Ephrin type-B receptor 2, ligand binding domain 19 - 196 IPR034238

Functions

Description
EC Number 2.7.-.- Transferring phosphorus-containing groups
Subcellular Localization
  • Mitochondrion membrane ; Single-pass membrane protein
  • Cytoplasm, cytosol
  • Cell membrane
PANTHER Family
PANTHER Subfamily
PANTHER Protein Class
PANTHER Pathway Category No pathway information available

3 GO annotations of cellular component

Name Definition
cytosol The part of the cytoplasm that does not contain organelles but which does contain other particulate matter, such as protein complexes.
mitochondrial membrane Either of the lipid bilayers that surround the mitochondrion and form the mitochondrial envelope.
plasma membrane The membrane surrounding a cell that separates the cell from its external environment. It consists of a phospholipid bilayer and associated proteins.

2 GO annotations of molecular function

Name Definition
ATP binding Binding to ATP, adenosine 5'-triphosphate, a universally important coenzyme and enzyme regulator.
kinase activity Catalysis of the transfer of a phosphate group, usually from ATP, to a substrate molecule.

5 GO annotations of biological process

Name Definition
cerebellar Purkinje cell layer morphogenesis The process in which the anatomical structure of the cerebellar Purkinje cell layer is generated and organized. The Purkinje cell layer lies just underneath the molecular layer of the cerebellar cortex. It contains the neuronal cell bodies of the Purkinje cells that are arranged side by side in a single layer. Candelabrum interneurons are vertically oriented between the Purkinje cells. Purkinje neurons are inhibitory and provide the output of the cerebellar cortex through axons that project into the white matter. Extensive dendritic trees from the Purkinje cells extend upward in a single plane into the molecular layer where they synapse with parallel fibers of granule cells.
glomerular basement membrane development The process whose specific outcome is the progression of the glomerular basement membrane over time, from its formation to the mature structure. The glomerular basement membrane is the basal laminal portion of the glomerulus which performs the actual filtration.
phosphorylation The process of introducing a phosphate group into a molecule, usually with the formation of a phosphoric ester, a phosphoric anhydride or a phosphoric amide.
podocyte development The process whose specific outcome is the progression of a glomerular visceral epithelial cell over time, from its formation to the mature structure. A glomerular visceral epithelial cell is a specialized epithelial cell that contains 'feet' that interdigitate with the 'feet' of other glomerular epithelial cells.
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
Q8NI60 COQ8A Atypical kinase COQ8A, mitochondrial Homo sapiens (Human) EV
Q96D53 COQ8B Atypical kinase COQ8B, mitochondrial Homo sapiens (Human) SS
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
Q18486 coq-8 Atypical kinase coq-8, mitochondrial Caenorhabditis elegans SS
Q9SBB2 ABC1 Protein ABC transporter 1, mitochondrial Arabidopsis thaliana (Mouse-ear cress) SS
Q5RGU1 coq8a Atypical kinase COQ8A, mitochondrial Danio rerio (Zebrafish) (Brachydanio rerio) SS
10 20 30 40 50 60
MLLSEVLQVL RGAGKVGAAF TSTQGEQLRL MACNSTFGAG MKAAAEAVEG VMGTVMGGGD
70 80 90 100 110 120
MTSKTDEFAG IEKWEEMDLD EAAKWSVASE MPPDFSSKDG RGETSETPVG AATGTIKGAG
130 140 150 160 170 180
WPAQNTRFLH VSASQHHFRF VHDSIVARLS PEDIQRAREA KQNIARPVRQ KLNERAKERK
190 200 210 220 230 240
VPATRISRLA NFGGLAVGLG IGAIAEVAKQ SFGGKRSEVG ALLDSPLLSE ANAERIVNTL
250 260 270 280 290 300
CKVRGAALKI GQMLSIQDNS FINPQLQKIF ERVRQSADFM PAWQMHKVLE EELGSGWREK
310 320 330 340 350 360
LSSIEEKPFA AASIGQVHHG VLPGGKEIAM KIQYPGVAES IHSDINNLMS VLKMSVVLPD
370 380 390 400 410 420
GLFADSSLEV LQRELAWECD YEREAKCAKR FRNLLKGDPV FVVPEVFDEL SARRVITMEL
430 440 450 460 470 480
VNGVPLDRCV DLDQETRNEI CFNILQLCLR ELFEFRFMQT DPNWSNFFYN SEQNKIFLLD
490 500 510 520 530 540
FGACRDYPEL FTDHYIEVVH AASVGDRATV LKKSKDLKFL TGFEAKAFED AHVEAVMILG
550 560 570 580 590 600
EAFASAEAFD FGTQSTTQRI QSLIPVMLRH RLTPPPEESY SLHRKMAGSF LICSKLKARF
610 620
SCRNMFLDVY NAYKRQQQER RSQV