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

260-510 (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 Q9SBB2

Entry ID Method Resolution Chain Position Source
AF-Q9SBB2-F1 Predicted AlphaFoldDB

57 variants for Q9SBB2

Variant ID(s) Position Change Description Diseaes Association Provenance
ENSVATH10506538 15 V>F No 1000Genomes
tmp_4_708719_A_G 23 Y>C No 1000Genomes
ENSVATH00452736 27 P>S No 1000Genomes
ENSVATH02613504 41 I>M No 1000Genomes
ENSVATH02613505 43 S>Y No 1000Genomes
tmp_4_708787_A_G 46 K>E No 1000Genomes
ENSVATH06416576 47 K>Q No 1000Genomes
tmp_4_708799_G_A 50 V>I No 1000Genomes
ENSVATH13874186 73 E>K No 1000Genomes
tmp_4_708896_G_A 82 G>E No 1000Genomes
tmp_4_708898_G_A 83 D>N No 1000Genomes
ENSVATH00452739 94 V>I No 1000Genomes
ENSVATH00452740 100 K>E No 1000Genomes
tmp_4_708956_G_A 102 S>N No 1000Genomes
ENSVATH06416578 104 K>R No 1000Genomes
ENSVATH10506540 105 I>F No 1000Genomes
ENSVATH00452741 113 N>S No 1000Genomes
tmp_4_708991_G_A 114 E>K No 1000Genomes
tmp_4_708995_T_C 115 F>S No 1000Genomes
tmp_4_709024_G_A 125 E>K No 1000Genomes
tmp_4_709028_G_A 126 G>D No 1000Genomes
tmp_4_709027_G_A 126 G>S No 1000Genomes
ENSVATH10506544 134 E>K No 1000Genomes
ENSVATH10506575 136 G>A No 1000Genomes
ENSVATH02613508 141 V>L No 1000Genomes
ENSVATH02613509 143 A>G No 1000Genomes
ENSVATH02613510 144 V>A No 1000Genomes
ENSVATH00452742 149 V>A No 1000Genomes
tmp_4_709100_C_T 150 T>M No 1000Genomes
ENSVATH00452743 151 E>V No 1000Genomes
ENSVATH02613511 160 P>A No 1000Genomes
ENSVATH02613511 160 P>T No 1000Genomes
tmp_4_709171_T_G 174 S>A No 1000Genomes
ENSVATH00452745 184 F>L No 1000Genomes
tmp_4_709356_C_T 207 P>S No 1000Genomes
ENSVATH06416582 211 D>A No 1000Genomes
ENSVATH13874189 217 S>P No 1000Genomes
tmp_4_709656_G_A 283 A>T No 1000Genomes
ENSVATH06416592 292 K>M No 1000Genomes
ENSVATH06416595 331 N>I No 1000Genomes
ENSVATH13874192 334 E>A No 1000Genomes
tmp_4_710198_G_A 382 R>H No 1000Genomes
tmp_4_710222_C_T 390 T>I No 1000Genomes
ENSVATH00452747 401 E>D No 1000Genomes
ENSVATH13874235 406 K>N No 1000Genomes
ENSVATH10506651 431 Y>F No 1000Genomes
ENSVATH06416604 489 R>Q No 1000Genomes
tmp_4_710700_G_A 493 A>T No 1000Genomes
ENSVATH06416608 498 D>E No 1000Genomes
ENSVATH06416609 504 E>K No 1000Genomes
ENSVATH10506654 540 G>D No 1000Genomes
tmp_4_710914_T_G 564 L>R No 1000Genomes
ENSVATH10506657 601 V>I No 1000Genomes
tmp_4_711028_A_G 602 Y>C No 1000Genomes
ENSVATH13874237 612 Q>R No 1000Genomes
ENSVATH06416615 614 P>L No 1000Genomes
tmp_4_711076_C_T 618 T>I No 1000Genomes

No associated diseases with Q9SBB2

2 regional properties for Q9SBB2

Type Name Position InterPro Accession
domain ABC1 atypical kinase-like domain 265 - 506 IPR004147
domain ADCK3-like domain 260 - 510 IPR034646

Functions

Description
EC Number 2.7.-.- Transferring phosphorus-containing groups
Subcellular Localization
  • Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side
PANTHER Family
PANTHER Subfamily
PANTHER Protein Class
PANTHER Pathway Category No pathway information available

2 GO annotations of cellular component

Name Definition
mitochondrial inner membrane The inner, i.e. lumen-facing, lipid bilayer of the mitochondrial envelope. It is highly folded to form cristae.
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.

4 GO annotations of biological process

Name Definition
chlorophyll catabolic process The chemical reactions and pathways resulting in the breakdown of chlorophyll, any compound of magnesium complexed in a porphyrin (tetrapyrrole) ring and which functions as a photosynthetic pigment, into less complex products.
cytochrome complex assembly The aggregation, arrangement and bonding together of a cytochrome complex. A cytochrome complex is a protein complex in which at least one of the proteins is a cytochrome, i.e. a heme-containing protein involved in catalysis of redox reactions.
response to UV-B Any process that results in a change in state or activity of a cell or an organism (in terms of movement, secretion, enzyme production, gene expression, etc.) as a result of a UV-B radiation stimulus. UV-B radiation (UV-B light) spans the wavelengths 280 to 315 nm.
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
Q18486 coq-8 Atypical kinase coq-8, mitochondrial Caenorhabditis elegans 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
MSSWSKSFTR LVNGVSLVAK EIYNQSPELQ RARNGDLEGL ITSSGKKALV AATDLVGLTS
70 80 90 100 110 120
GKLRELSIRR SKEPSVVYFD EGDNKDNVVA TPEVRSVNTK PSEKIVDSKV LRNEFSDEVK
130 140 150 160 170 180
NSNREGEIKV SGVEAGEIIG VRAVESGTVT EASSPSEVVP PVKRRRPRER KVPSTPMARA
190 200 210 220 230 240
YGFFNLGAAL AWGAVKESTY RMVNGTPMTP DNQPALSSLM SKENAERLAL GLCEMRGAAL
250 260 270 280 290 300
KVGQMLSIQD ESLVPAPILN ALEYVRQGAD VMPRSQLNPV LDAELGSNWQ SKLTSFDYEP
310 320 330 340 350 360
LAAASIGQVH RAVTKDGLEV AMKIQYPGVA NSIESDIENV RRLLNYTNLI PKGLFLDRAI
370 380 390 400 410 420
KVAKEELAQE CDYEIEAVSQ KRFRDLLSDT PGFYVPLVVD ETSSKKILTT ELISGIPIDK
430 440 450 460 470 480
VALLDQKTRD YVGRKMLELT LKELFVFRFM QTDPNWGNFL YNEATKTINL IDFGAARDYP
490 500 510 520 530 540
KKFVDDYLRM VMACAEKDSE GVIEMSRRLG FLTGDESDVM LDAHVQAGFI VGLPFAEPGG
550 560 570 580 590 600
YAFRTNNIAS SISNLGATML KHRLSPPPDE AYSLHRKLSG AFLACIKLGA TVPCRDLLLQ
610 620
VYNKYQFDDE PQDPVVATSS VSS