Atypical kinase COQ8A, mitochondrial

Details

Name

Atypical kinase COQ8A, mitochondrial

Synonyms

• 2.7.-.-
• aarF domain-containing protein kinase 3
• ADCK3
• CABC1
• Chaperone activity of bc1 complex-like
• Chaperone-ABC1-like
• Coenzyme Q protein 8A

Gene Name

COQ8A

Organism

Humans

Amino acid sequence

>lcl|BSEQ0051640|Atypical kinase COQ8A, mitochondrial
MAAILGDTIMVAKGLVKLTQAAVETHLQHLGIGGELIMAARALQSTAVEQIGMFLGKVQG
QDKHEEYFAENFGGPEGEFHFSVPHAAGASTDFSSASAPDQSAPPSLGHAHSEGPAPAYV
ASGPFREAGFPGQASSPLGRANGRLFANPRDSFSAMGFQRRFFHQDQSPVGGLTAEDIEK
ARQAKARPENKQHKQTLSEHARERKVPVTRIGRLANFGGLAVGLGFGALAEVAKKSLRSE
DPSGKKAVLGSSPFLSEANAERIVRTLCKVRGAALKLGQMLSIQDDAFINPHLAKIFERV
RQSADFMPLKQMMKTLNNDLGPNWRDKLEYFEERPFAAASIGQVHLARMKGGREVAMKIQ
YPGVAQSINSDVNNLMAVLNMSNMLPEGLFPEHLIDVLRRELALECDYQREAACARKFRD
LLKGHPFFYVPEIVDELCSPHVLTTELVSGFPLDQAEGLSQEIRNEICYNILVLCLRELF
EFHFMQTDPNWSNFFYDPQQHKVALLDFGATREYDRSFTDLYIQIIRAAADRDRETVRAK
SIEMKFLTGYEVKVMEDAHLDAILILGEAFASDEPFDFGTQSTTEKIHNLIPVMLRHRLV
PPPEETYSLHRKMGGSFLICSKLKARFPCKAMFEEAYSNYCKRQAQQ

Number of residues

647

Molecular Weight

71949.52

Theoretical pI

Not Available

GO Classification

Functions

ADP binding / ATP binding / kinase activity

Processes

phosphorylation / ubiquinone biosynthetic process

Components

integral component of membrane / mitochondrion

General Function

Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration (PubMed:25498144, PubMed:21296186, PubMed:25540914, PubMed:27499294). Its substrate specificity is unclear: does not show any protein kinase activity (PubMed:25498144, PubMed:27499294). Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates (PubMed:25498144, PubMed:27499294). Shows an unusual selectivity for binding ADP over ATP (PubMed:25498144).

Specific Function

Adp binding

Pfam Domain Function

• ABC1 (PF03109)

Transmembrane Regions

214-230

Cellular Location

Mitochondrion

Gene sequence

>lcl|BSEQ0051641|Atypical kinase COQ8A, mitochondrial (COQ8A)
ATGGCTGCCATATTGGGAGACACCATCATGGTGGCTAAAGGCCTTGTCAAGCTGACCCAG
GCGGCCGTGGAAACCCACCTGCAGCACTTGGGCATCGGAGGGGAGCTGATCATGGCGGCC
AGGGCCCTGCAGTCCACGGCTGTGGAGCAGATTGGCATGTTCTTGGGGAAGGTGCAGGGT
CAGGATAAACATGAAGAATATTTTGCTGAGAACTTCGGCGGCCCAGAAGGGGAGTTCCAC
TTCTCAGTCCCGCATGCAGCCGGAGCCTCCACAGACTTCTCTTCAGCCTCCGCTCCCGAC
CAGTCAGCGCCCCCATCCCTGGGTCATGCCCACAGCGAGGGCCCAGCTCCTGCCTACGTG
GCCAGTGGACCCTTTAGAGAAGCCGGGTTCCCCGGCCAGGCCTCCTCCCCTCTGGGCAGG
GCCAACGGGAGGCTCTTTGCAAACCCCAGAGACTCATTCTCTGCCATGGGCTTTCAGCGA
AGGTTCTTCCACCAGGACCAATCCCCTGTTGGGGGCCTCACAGCCGAGGACATTGAGAAG
GCCCGGCAGGCTAAGGCTCGCCCCGAGAACAAGCAGCACAAACAGACGCTCAGCGAGCAT
GCCCGGGAGCGGAAGGTGCCTGTGACGAGGATTGGCCGGCTGGCCAACTTCGGAGGTCTG
GCCGTGGGCCTGGGCTTCGGGGCACTGGCAGAGGTCGCCAAGAAGAGCCTGCGCTCCGAG
GACCCCTCAGGGAAGAAGGCCGTGCTGGGTTCCAGTCCTTTCCTGTCCGAGGCCAATGCA
GAGCGGATCGTGCGCACGCTCTGCAAGGTGCGTGGTGCGGCACTCAAGCTGGGCCAGATG
CTGAGCATCCAGGATGATGCCTTTATCAACCCCCACCTGGCTAAGATCTTCGAGCGGGTG
CGGCAGAGCGCGGACTTCATGCCACTGAAGCAGATGATGAAAACTCTCAACAACGACCTG
GGCCCCAACTGGCGGGACAAGTTGGAATACTTCGAGGAGCGGCCCTTCGCCGCCGCATCC
ATTGGGCAGGTGCACTTGGCCCGAATGAAGGGCGGCCGCGAGGTGGCCATGAAGATCCAG
TACCCTGGCGTGGCCCAGAGCATCAACAGTGATGTCAACAACCTCATGGCCGTGTTGAAC
ATGAGCAACATGCTTCCAGAAGGCCTGTTCCCCGAGCACCTGATCGACGTGCTGAGGCGG
GAGCTGGCCCTGGAGTGTGACTACCAGCGAGAGGCCGCCTGTGCCCGCAAGTTCAGGGAC
CTGCTGAAGGGCCACCCCTTCTTCTATGTGCCTGAGATTGTGGATGAGCTCTGCAGCCCA
CATGTGCTGACCACAGAGCTGGTGTCTGGCTTCCCCCTGGACCAGGCCGAAGGGCTCAGC
CAGGAGATTCGGAACGAGATCTGCTACAACATCCTGGTTCTGTGCCTGAGGGAGCTGTTC
GAGTTCCACTTCATGCAAACAGACCCCAACTGGTCCAACTTCTTCTATGACCCCCAGCAG
CACAAGGTGGCTCTTTTGGATTTTGGGGCAACGCGGGAATATGACAGATCCTTCACCGAC
CTCTACATTCAGATCATCAGGGCTGCTGCCGACAGGGACAGGGAGACTGTGCGGGCGAAA
TCCATAGAGATGAAGTTCCTCACCGGCTACGAGGTCAAGGTCATGGAAGACGCCCACTTG
GATGCCATCCTCATCCTGGGGGAGGCCTTCGCCTCTGATGAGCCTTTTGATTTTGGCACT
CAGAGCACCACCGAGAAGATCCACAACCTGATTCCCGTCATGCTGAGGCACCGTCTCGTC
CCCCCACCCGAGGAAACCTACTCCCTGCACAGGAAGATGGGGGGCTCCTTCCTCATCTGC
TCCAAGCTGAAGGCCCGCTTCCCCTGCAAGGCCATGTTCGAGGAGGCCTACAGCAACTAC
TGCAAGAGGCAGGCCCAGCAGTAG

Chromosome Location

1

Locus

1q42.13

External Identifiers

Resource	Link
UniProtKB ID	Q8NI60
UniProtKB Entry Name	COQ8A_HUMAN
HGNC ID	HGNC:16812

General References

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Drug Relations

Drug Relations

DrugBank ID	Name	Drug group	Pharmacological action?	Actions	Details
DB12010	Fostamatinib	approved, investigational	unknown	inhibitor	Details