Q6AY19

SS
Gene name
Coq8b (Adck4)
Protein name
Atypical kinase COQ8B, mitochondrial
Names
EC 2.7.-.- , AarF domain-containing protein kinase 4 , Coenzyme Q protein 8B
Species
Rattus norvegicus (Rat)
KEGG Pathway
rno:308453
EC number
2.7.-.-: Transferring phosphorus-containing groups
Protein Class

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)

151-154 (KxGQ motif) SS

Target domain

170-420 (Activator of bc1 complex, ABC1, kinases, also called aarF domain containing kinase 3)

Relief mechanism

PTM

Assay

Accessory elements

No accessory elements

References

Autoinhibited structure

Activated structure

1 structures for Q6AY19

Entry ID Method Resolution Chain Position Source
AF-Q6AY19-F1 Predicted AlphaFoldDB

1 variants for Q6AY19

Variant ID(s) Position Change Description Diseaes Association Provenance
rs3318949766 352 R>Q No EVA

No associated diseases with Q6AY19

7 regional properties for Q6AY19

Type Name Position InterPro Accession
domain Zinc finger, RING-type 133 - 181 IPR001841
domain IBR domain 203 - 264 IPR002867-1
domain IBR domain 272 - 336 IPR002867-2
domain Zinc finger, C3HC4 RING-type 133 - 166 IPR018957
domain TRIAD supradomain 129 - 340 IPR044066
domain Ariadne domain 350 - 474 IPR045840
domain E3 ubiquitin-protein ligase ARIH1-like, UBA-like domain 59 - 99 IPR048962

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
Q566J8 Coq8b Atypical kinase COQ8B, mitochondrial Mus musculus (Mouse) SS
Q5BJQ0 Coq8a Atypical kinase COQ8A, 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 TTCGPLGRAV RLPCAGALRL RPHWWGPCRD CLAQRVHQDQ PGRGLTEDEI
70 80 90 100 110 120
RRAREARLRK APRPQLSDRS RERKVPASRI SRLASFGGLA VGLGLGALAE VTKKSLPGGS
130 140 150 160 170 180
LQHEGSSPFL TEANAERIVQ TLCTVRGAAL KIGQMLSIQD NSLISPQLQR VFERVRQSAD
190 200 210 220 230 240
FMPRWQMMKV LEEELGKDWQ DKVASLEEVP FAAASIGQVH QGVLKDGTEV AVKIQYPGVA
250 260 270 280 290 300
ESIQSDVQNL LALLKMSVGL PEGLFAEQSL QTLQQELAWE CDYRREAACA QTFKKLLADD
310 320 330 340 350 360
PFFRVPAVVE ELCTTRVLGM ELAGGIPLDQ CQGLSQDIRN QICFQLLRLC LRELFEFRFM
370 380 390 400 410 420
QTDPNWANFL YDASSHKVTL LDFGASRAFG TEFTDHYIEV VKAAADGDRD RVLQKSQDLK
430 440 450 460 470 480
FLTGFETKAF SDAHVEAVMI LGEPFAASGS YDFGAGETAR RIQGLIPVLL RHRLRPPPEE
490 500 510 520
TYALHRKLAG AFLACARLHA HIACRDLFQD TYHRYWASRQ TLPLPAAS