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

175-425 (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 Q566J8

Entry ID Method Resolution Chain Position Source
AF-Q566J8-F1 Predicted AlphaFoldDB

No variants for Q566J8

Variant ID(s) Position Change Description Diseaes Association Provenance
No variants for Q566J8

No associated diseases with Q566J8

4 regional properties for Q566J8

Type Name Position InterPro Accession
domain Ras-like guanine nucleotide exchange factor, N-terminal 49 - 175 IPR000651
domain Ras guanine-nucleotide exchange factors catalytic domain 197 - 433 IPR001895
domain EF-hand domain 466 - 501 IPR002048
domain Protein kinase C-like, phorbol ester/diacylglycerol-binding domain 540 - 597 IPR002219

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

4 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.
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.
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.

3 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.
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.
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
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
A3QJU3 coq8b Atypical kinase COQ8B, mitochondrial Danio rerio (Zebrafish) (Brachydanio rerio) SS
10 20 30 40 50 60
MWLELGAMLR RTCGPLGRAV RLPCGGALGP RPHWWGPCRS CLAQSVHQDQ PGRGLSEDDI
70 80 90 100 110 120
RRAREARLRK APRPQLSDRS RERKVPASRI SRLASFGGLA VGLGLGALAE VTKKSLPGGS
130 140 150 160 170 180
LQHEGVSGLG SSPFLSEANA ERIVQTLCTV RGAALKIGQM LSIQDNSFIS PQLQRIFERV
190 200 210 220 230 240
RQSADFMPRW QMMRVLEEEL GKDWQDKVAS LEEVPFAAAS IGQVHQGLLK DGTEVAVKIQ
250 260 270 280 290 300
YPGVAQSIQS DVENLLALLK MSVGLPEGLF AEQSLQTLQQ ELAWECDYRR EAACAQTFRK
310 320 330 340 350 360
LLADDPFFRV PAVVQELCTT RVLGMELAGG IPLDQCQGLS QDIRNQICFQ LLRLCLRELF
370 380 390 400 410 420
EFRFMQTDPN WANFLYDASS HQVTLLDFGA SRAFGTEFTD HYIEVVKAAA DGDRDRVLQK
430 440 450 460 470 480
SQDLKFLTGF ETKAFSDAHV EAVMILGEPF AASGPYDFGA GETARRIQGL IPVLLRHRLR
490 500 510 520 530
PPPEETYALH RKLAGAFLAC ARLHAHIACR DLFQDTYHRY WASRQTLPLP AAS