Q29RI0

SS
Gene name
COQ8A
Protein name
Atypical kinase COQ8A, mitochondrial
Names
Chaperone activity of bc1 complex-like, Chaperone-ABC1-like, Coenzyme Q protein 8A, aarF domain-containing protein kinase 3
Species
Bos taurus (Bovine)
KEGG Pathway
bta:536925
EC number
Protein Class

Descriptions

UbiB protein kinase-like (PKL) family is wide-spread, comprising one-quarter of microbial PKLs and five human homologs. COQ8A is a mammalian UbiB protein associated with ubiquinone (CoQ) biosynthesis. A human homolog lacks canonical protein kinase activity in trans. Instead, it has ATPase activity and interacts with lipid CoQ intermediates. 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.

Autoinhibitory domains (AIDs)

276-279 (KxGQ motif) SS

Target domain

295-545 (ADCK3-like domain)

Relief mechanism

PTM

Assay

Accessory elements

No accessory elements

References

Autoinhibited structure

Activated structure

1 structures for Q29RI0

Entry ID Method Resolution Chain Position Source
AF-Q29RI0-F1 Predicted AlphaFoldDB

246 variants for Q29RI0

Variant ID(s) Position Change Description Diseaes Association Provenance
rs483055498 6 G>V No Ensembl
rs459197187 7 D>E No Ensembl
rs445476067 7 D>V No Ensembl
rs467832686 14 G>A No Ensembl
rs447585364 14 G>C No Ensembl
rs450238497 15 F>C No Ensembl
rs450238497 15 F>Y No Ensembl
rs469140699 16 V>G No Ensembl
rs437812298 20 Q>R No Ensembl
rs471678441 23 V>G No Ensembl
rs433918997 30 L>P No Ensembl
rs453633434 32 L>F No Ensembl
rs473639735 35 E>A No Ensembl
rs442456914 36 L>H No Ensembl
rs476576982 40 V>G No Ensembl
rs438993521 42 A>S No Ensembl
rs459190514 51 V>A No Ensembl
rs447996389 53 M>L No Ensembl
rs481432694 54 V>D No Ensembl
rs461321020 54 V>F No Ensembl
rs450175255 55 F>V No Ensembl
rs439056872 92 D>Y No Ensembl
rs459256012 100 E>* No Ensembl
rs478956331 103 P>L No Ensembl
rs110822050 110 A>T No Ensembl
rs461382460 119 Y>D No Ensembl
rs450192054 124 P>T No Ensembl
rs469123804 127 E>A No Ensembl
rs441752932 129 G>R No Ensembl
rs441752932 129 G>R No Ensembl
rs465273629 133 Q>E No Ensembl
rs436031042 141 V>E No Ensembl
rs436031042 141 V>G No Ensembl
rs432561414 144 R>M No Ensembl
rs472876948 145 L>H No Ensembl
rs452667605 145 L>I No Ensembl
rs452667605 145 L>V No Ensembl
rs441574898 147 A>T No Ensembl
rs461323063 149 P>T No Ensembl
rs475021837 150 R>I No Ensembl
rs443660006 151 D>G No Ensembl
rs458616427 160 R>G No Ensembl
rs478700889 161 R>M No Ensembl
rs467175875 162 V>G No Ensembl
rs435967860 163 Y>D No Ensembl
rs449652447 164 H>N No Ensembl
rs469893681 164 H>P No Ensembl
rs432102286 165 Q>H No Ensembl
rs452659908 167 Q>L No Ensembl
rs435047922 168 S>L No Ensembl
rs472815597 168 S>P No Ensembl
rs455241677 169 S>A No Ensembl
rs443617996 175 A>P No Ensembl
rs463765586 176 E>V No Ensembl
rs471098252 178 I>M No Ensembl
rs439727508 179 E>V No Ensembl
rs458557476 180 K>Q No Ensembl
rs481423269 183 Q>E No Ensembl
rs469798319 193 H>P No Ensembl
rs469798319 193 H>R No Ensembl
rs432035137 194 K>Q No Ensembl
rs466330958 195 Q>P No Ensembl
rs461830250 197 L>P No Ensembl
rs482426392 198 S>G No Ensembl
rs450982917 198 S>R No Ensembl
rs445620802 204 R>Q No Ensembl
rs458379693 206 V>G No Ensembl
rs466768751 217 F>V No Ensembl
rs435179959 228 A>D No Ensembl
rs466476243 228 A>P No Ensembl
rs475288040 235 K>N No Ensembl
rs437691560 241 D>E No Ensembl
rs451759161 242 P>T No Ensembl
rs466005130 260 A>T No Ensembl
rs434635913 266 T>P No Ensembl
rs482498250 286 D>V No Ensembl
rs42605795 287 A>P No Ensembl
rs444897124 290 N>T No Ensembl
rs465533103 299 R>Q No Ensembl
rs447906108 302 Q>K No Ensembl
rs468046690 305 D>A No Ensembl
rs476588667 314 K>Q No Ensembl
rs438361543 315 T>P No Ensembl
rs438361543 315 T>S No Ensembl
rs458584364 316 L>F No Ensembl
rs478710441 317 N>K No Ensembl
rs447404094 319 D>A No Ensembl
rs461494596 319 D>E No Ensembl
rs481623124 321 G>C No Ensembl
rs477776941 324 W>* No Ensembl
rs470454888 324 W>* No Ensembl
rs477776941 324 W>C No Ensembl
rs450363322 324 W>R No Ensembl
rs445963310 326 D>A No Ensembl
rs466205777 326 D>E No Ensembl
rs445963310 326 D>G No Ensembl
rs434832754 327 K>E No Ensembl
rs454887418 327 K>M No Ensembl
rs468547260 327 K>N No Ensembl
rs434832754 327 K>Q No Ensembl
rs436147494 328 L>M No Ensembl
rs456203241 328 L>R No Ensembl
rs451979057 329 E>D No Ensembl
rs438699681 329 E>G No Ensembl
rs472230799 330 Y>D No Ensembl
rs440900341 331 F>L No Ensembl
rs481143123 332 E>* No Ensembl
rs443828682 332 E>A No Ensembl
rs463981752 332 E>D No Ensembl
rs443828682 332 E>G No Ensembl
rs446329027 333 E>D No Ensembl
rs477731209 333 E>Q No Ensembl
rs466084083 334 R>G No Ensembl
rs479606774 334 R>Q No Ensembl
rs448441567 335 P>A No Ensembl
rs468485491 335 P>R No Ensembl
rs437292682 336 F>L No Ensembl
rs470018088 337 A>P No Ensembl
rs470018088 337 A>S No Ensembl
rs470018088 337 A>T No Ensembl
rs452387039 338 A>D No Ensembl
rs452387039 338 A>G No Ensembl
rs432223675 338 A>T No Ensembl
rs472520912 339 A>P No Ensembl
rs472520912 339 A>S No Ensembl
rs472520912 339 A>T No Ensembl
rs454559620 340 S>P No Ensembl
rs474668083 341 I>L No Ensembl
rs463870037 342 G>A No Ensembl
rs443369558 342 G>R No Ensembl
rs443369558 342 G>W No Ensembl
rs477649763 343 Q>E No Ensembl
rs42605794 343 Q>H No Ensembl
rs439832120 344 V>G No Ensembl
rs479945989 345 H>L No Ensembl
rs460048814 345 H>N No Ensembl
rs448376088 345 H>Q No Ensembl
rs461994589 346 L>P No Ensembl
rs450975029 347 A>S No Ensembl
rs432144269 348 R>H No Ensembl
rs434688410 350 K>R No Ensembl
rs1117260211 351 G>V No Ensembl
rs454497996 353 R>G No Ensembl
rs474605149 355 V>A No Ensembl
rs474605149 355 V>G No Ensembl
rs471474110 362 P>T No Ensembl
rs440268646 367 S>R No Ensembl
rs448914284 376 M>R No Ensembl
rs432473392 390 F>C No Ensembl
rs451348708 399 R>Q No Ensembl
rs440203592 401 E>V No Ensembl
rs453877720 405 E>* No Ensembl
rs473934898 406 C>G No Ensembl
rs462567797 412 A>P No Ensembl
rs462567797 412 A>S No Ensembl
rs482760516 413 A>V No Ensembl
rs459093729 416 R>C No Ensembl
rs459093729 416 R>S No Ensembl
rs479195116 417 R>W No Ensembl
rs447930403 418 F>S No Ensembl
rs482410961 420 E>* No Ensembl
rs449831624 424 D>E No Ensembl
rs469990474 427 F>C No Ensembl
rs432198511 436 E>K No Ensembl
rs466112341 441 H>L No Ensembl
rs434345812 442 V>E No Ensembl
rs468261665 444 T>P No Ensembl
rs457493964 445 T>M No Ensembl
rs436904321 445 T>S No Ensembl
rs439806222 446 E>D No Ensembl
rs471183535 446 E>Q No Ensembl
rs473529231 448 V>E No Ensembl
rs441949537 449 S>P No Ensembl
rs462134302 450 G>C No Ensembl
rs444495218 451 F>L No Ensembl
rs482342401 451 F>S No Ensembl
rs463523006 453 L>M No Ensembl
rs477188282 453 L>R No Ensembl
rs445892484 455 Q>H No Ensembl
rs466048884 463 I>T No Ensembl
rs378388510 477 R>W No Ensembl
rs441856805 490 N>T No Ensembl
rs461995366 495 F>V No Ensembl
rs435779139 507 D>A No Ensembl
rs461633442 557 D>A No Ensembl
rs481809053 558 A>G No Ensembl
rs450033861 559 H>Q No Ensembl
rs477267894 565 I>L No Ensembl
rs446095980 565 I>M No Ensembl
rs466150137 566 L>R No Ensembl
rs433749308 567 G>E No Ensembl
rs447429376 573 E>D No Ensembl
rs436305090 578 F>S No Ensembl
rs467660782 578 F>V No Ensembl
rs456063844 584 T>P No Ensembl
rs431987095 586 K>N No Ensembl
rs476211201 586 K>T No Ensembl
rs452222293 589 N>T No Ensembl
rs472313430 593 I>N No Ensembl
rs441385815 595 L>V No Ensembl
rs455059640 596 K>Q No Ensembl
rs443904383 600 V>A No Ensembl
rs443904383 600 V>G No Ensembl
rs459421392 600 V>L No Ensembl
rs439592446 602 P>R No Ensembl
rs467542829 604 E>A No Ensembl
rs467542829 604 E>G No Ensembl
rs447415550 604 E>Q No Ensembl
rs481319369 605 E>G No Ensembl
rs449994000 606 T>A No Ensembl
rs470123385 606 T>N No Ensembl
rs470123385 606 T>S No Ensembl
rs431929601 607 Y>H No Ensembl
rs431929601 607 Y>N No Ensembl
rs452161191 607 Y>S No Ensembl
rs434500978 608 S>F No Ensembl
rs465829231 608 S>P No Ensembl
rs455078603 609 L>P No Ensembl
rs475199304 610 H>P No Ensembl
rs443864827 611 R>G No Ensembl
rs457455755 611 R>T No Ensembl
rs471145813 612 K>E No Ensembl
rs479905339 613 M>K No Ensembl
rs479905339 613 M>R No Ensembl
rs459606251 613 M>V No Ensembl
rs481272362 616 S>A No Ensembl
rs481272362 616 S>P No Ensembl
rs449930944 617 F>L No Ensembl
rs470059824 618 L>F No Ensembl
rs477313908 618 L>P No Ensembl
rs445634651 619 I>F No Ensembl
rs445634651 619 I>L No Ensembl
rs434416504 620 C>F No Ensembl
rs465735773 620 C>R No Ensembl
rs454540080 621 S>C No Ensembl
rs468325203 622 K>Q No Ensembl
rs457392642 623 L>P No Ensembl
rs457392642 623 L>R No Ensembl
rs471083093 624 K>E No Ensembl
rs433459094 625 A>D No Ensembl
rs453143135 627 F>V No Ensembl
rs473376375 637 Y>D No Ensembl
rs442054021 638 S>R No Ensembl
rs462196060 640 Y>* No Ensembl
rs475984413 643 R>T No Ensembl
rs474712983 645 A>S No Ensembl

No associated diseases with Q29RI0

15 regional properties for Q29RI0

Type Name Position InterPro Accession
domain Sema domain 20 - 509 IPR001627
repeat Plexin repeat 511 - 552 IPR002165-1
repeat Plexin repeat 658 - 701 IPR002165-2
repeat Plexin repeat 807 - 851 IPR002165-3
domain IPT domain 860 - 955 IPR002909-1
domain IPT domain 956 - 1041 IPR002909-2
domain IPT domain 1043 - 1143 IPR002909-3
domain IPT domain 1145 - 1240 IPR002909-4
domain Plexin, cytoplasmic RasGAP domain 1315 - 1862 IPR013548
domain PSI domain 511 - 561 IPR016201-1
domain PSI domain 658 - 705 IPR016201-2
domain PSI domain 806 - 859 IPR016201-3
domain Plexin, TIG domain 1 566 - 657 IPR041019
domain Plexin, TIG domain 2 712 - 805 IPR041362
domain Plexin, cytoplasmic RhoGTPase-binding domain 1484 - 1596 IPR046800

Functions

Description
EC Number
Subcellular Localization
  • Mitochondrion
  • Membrane ; Single-pass membrane protein
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.
integral component of membrane The component of a membrane consisting of the gene products and protein complexes having at least some part of their peptide sequence embedded in the hydrophobic region of the membrane.
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.

3 GO annotations of molecular function

Name Definition
ADP binding Binding to ADP, adenosine 5'-diphosphate.
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.

2 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.
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
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
MAAMLGDAIM LIKGFVKLTQ AAVETHLQHL GLSGELLMAV RALQSTATEQ VGMVFGQVQG
70 80 90 100 110 120
QETPEEYYSE SLDDPEGAFH FSGMRAESAS ADVSAASSPE QSPPPWAHAA GSEGPAPAYV
130 140 150 160 170 180
ASAPFREGGV LGQAASPLGR VNGRLFASPR DPFSAPGLQR RVYHQDQSSM GGLTAEDIEK
190 200 210 220 230 240
ARQAKARPES KPHKQALSEH ARERKVPVTR IGRLANFGGL AVGLGFGALA EVAKKSLRPD
250 260 270 280 290 300
DPSGKKAVLD SSPFLSEANA ERIVRTLCKV RGAALKLGQM LSIQDDAFIN PHLAKIFDRV
310 320 330 340 350 360
RQSADFMPLK QMMKTLNNDL GPNWRDKLEY FEERPFAAAS IGQVHLARLK GGREVAMKIQ
370 380 390 400 410 420
YPGVAQSINS DVNNLMTVLN MSNMLPEGLF PEHLIDVLRR ELALECDYQR EAACARRFRE
430 440 450 460 470 480
LLKDHPFFYV PEIVDELCSP HVLTTELVSG FPLDQAEGLS QEIRNEICYN ILVLCLRELF
490 500 510 520 530 540
EFQFMQTDPN WSNFFYDPEL HKVALLDFGA TREFDRSFTD LYIQIIRAAA NQDREAVLKK
550 560 570 580 590 600
SIEMKFLTGY EVKAMEDAHL DAILILGEAF ASEEPFDFGT QSTTEKIHNL IPIMLKHRLV
610 620 630 640
PPPEETYSLH RKMGGSFLIC SKLKARFPCK AMFEEAYSNY CRRQAEQQ