Descriptions

COQ8 is a atypical kinase involved in coenzyme Q biosynthesis. A human homolgous protein is known to be autoinhibited by the intramolecular interaction between the KxGQ motif in N-termianl region and the binding site of the catalytic domain in the protein.

Autoinhibitory domains (AIDs)

Target domain

153-403 (ADCK3-like domain)

Relief mechanism

PTM

Assay

Accessory elements

No accessory elements

Autoinhibited structure

Activated structure

1 structures for P27697

Entry ID Method Resolution Chain Position Source
AF-P27697-F1 Predicted AlphaFoldDB

7 variants for P27697

Variant ID(s) Position Change Description Diseaes Association Provenance
s07-284550 35 I>V No SGRP
s07-284588 47 K>N No SGRP
s07-284596 50 E>A No SGRP
s07-284612 55 R>S No SGRP
s07-285072 209 S>G No SGRP
s07-285259 271 R>K No SGRP
s07-285468 341 K>Q No SGRP

No associated diseases with P27697

2 regional properties for P27697

Type Name Position InterPro Accession
domain ABC1 atypical kinase-like domain 158 - 398 IPR004147
domain ADCK3-like domain 153 - 403 IPR034646

Functions

Description
EC Number
Subcellular Localization
  • Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side
PANTHER Family
PANTHER Subfamily
PANTHER Protein Class
PANTHER Pathway Category No pathway information available

3 GO annotations of cellular component

Name Definition
extrinsic component of mitochondrial inner membrane The component of mitochondrial inner membrane consisting of gene products and protein complexes that are loosely bound to one of its surfaces, but not integrated into the hydrophobic region.
mitochondrial matrix The gel-like material, with considerable fine structure, that lies in the matrix space, or lumen, of a mitochondrion. It contains the enzymes of the tricarboxylic acid cycle and, in some organisms, the enzymes concerned with fatty acid oxidation.
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.

5 GO annotations of molecular function

Name Definition
ATP binding Binding to ATP, adenosine 5'-triphosphate, a universally important coenzyme and enzyme regulator.
ATP hydrolysis activity Catalysis of the reaction: ATP + H2O = ADP + H+ phosphate. ATP hydrolysis is used in some reactions as an energy source, for example to catalyze a reaction or drive transport against a concentration gradient.
kinase activity Catalysis of the transfer of a phosphate group, usually from ATP, to a substrate molecule.
lipid binding Binding to a lipid.
protein kinase activity Catalysis of the phosphorylation of an amino acid residue in a protein, usually according to the reaction: a protein + ATP = a phosphoprotein + ADP.

4 GO annotations of biological process

Name Definition
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.
protein autophosphorylation The phosphorylation by a protein of one or more of its own amino acid residues (cis-autophosphorylation), or residues on an identical protein (trans-autophosphorylation).
ubiquinone biosynthetic process The chemical reactions and pathways resulting in the formation of ubiquinone, a lipid-soluble electron-transporting coenzyme.
ubiquinone-6 biosynthetic process The chemical reactions and pathways resulting in the formation of ubiquinone-6.

11 homologous proteins in AiPD

UniProt AC Gene Name Protein Name Species Evidence Code
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
Q566J8 Coq8b Atypical kinase COQ8B, mitochondrial Mus musculus (Mouse) SS
Q60936 Coq8a Atypical kinase COQ8A, mitochondrial Mus musculus (Mouse) SS
Q6AY19 Coq8b Atypical kinase COQ8B, mitochondrial Rattus norvegicus (Rat) 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
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
MVTNMVKLRN LRRLYCSSRL LRTIQNGRIS SVSSISLSKK YTTKSAKEGE ENVERKHEEE
70 80 90 100 110 120
KKDTLKSSSV PTSRISRLFH YGSLAAGVGM NAAAKGISEV AKGNSPTWKS LILSDSNIDR
130 140 150 160 170 180
ITNKFSKMRG VALKIGQLLS FQDEKVLPKE LYEILSRVQN SANHMPQRQL EKVMAKELGA
190 200 210 220 230 240
NWKTKFSKFD KIPMAAASIG QVHAAELPSG QRVVVKIQYP GVKESIDSDL NSLLMLLTAS
250 260 270 280 290 300
SLLPKGLFLD KTIANARTEL KWECDYNREA RALQKFEALL KDDPAFEVPH VFPEYTTDNI
310 320 330 340 350 360
ITMTRMEGTE IMKLPKASQE TKNFISENIM RLCLEEIATF KYMQTDPNWA NFLYNGRTKK
370 380 390 400 410 420
IELLDFGASR PFAEDFILKY RKLLTYATLR DRKGAYEMSV QLGYLTGLES QSMKDAHVDS
430 440 450 460 470 480
VLTLGEPFRG DVDKSFDFKD QTVSDRIRGN IGLMLNERLC PPPEETYSLH RKFSGIFLLC
490 500
ARMGASVHCA KLFKEIFAYK V