COQ5

Protein identityUniProt · HPA

UniProt accession

Q5HYK3

Gene

COQ5

Ensembl

ENSG00000110871

Chromosome

12

Canonical length

327 aa

Protein class

Disease related genes, Enzymes, Human disease related genes, Metabolic proteins, Potential drug targets, Predicted intracellular proteins

Subcellular location

Nucleoli,Mitochondria

OverviewNCBI Gene

Enables 2-methoxy-6-polyprenyl-1,4-benzoquinol methyltransferase activity. Involved in methylation and ubiquinone biosynthetic process. Located in mitochondrial inner membrane and mitochondrial matrix. Part of ubiquinone biosynthesis complex. Implicated in primary coenzyme Q10 deficiency 9. [provided by Alliance of Genome Resources, Jul 2025]

Canonical amino-acid sequenceUniProt

327 residues, UniProt reviewed canonical sequence.

>Q5HYK3|COQ5
     1  MAAPGSCALW SYCGRGWSRA MRGCQLLGLR SSWPGDLLSA RLLSQEKRAA ETHFGFETVS
    61  EEEKGGKVYQ VFESVAKKYD VMNDMMSLGI HRVWKDLLLW KMHPLPGTQL LDVAGGTGDI
   121  AFRFLNYVQS QHQRKQKRQL RAQQNLSWEE IAKEYQNEED SLGGSRVVVC DINKEMLKVG
   181  KQKALAQGYR AGLAWVLGDA EELPFDDDKF DIYTIAFGIR NVTHIDQALQ EAHRVLKPGG
   241  RFLCLEFSQV NNPLISRLYD LYSFQVIPVL GEVIAGDWKS YQYLVESIRR FPSQEEFKDM
   301  IEDAGFHKVT YESLTSGIVA IHSGFKL

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against COQ5 can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Other membrane

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.33

Highest tissue expression

92 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about COQ5.

Disease | AllUniProt

Conditions COQ5 is implicated in, by any mechanism.

• Coenzyme Q10 deficiency, primary, 9 (COQ10D9) MIM:619028

Disease | GeneticClinVar

2 pathogenic / likely-pathogenic of 72 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Coenzyme q10 deficiency, primary, 9

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.95

gnomAD pLI

0

gnomAD missense Z

-0.36

DepMap mean gene effect

-0.23

DepMap dependency class

selective

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 5% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• methylation
• ubiquinone biosynthetic process

Molecular functions

• 2-methoxy-6-polyprenyl-1
• 4-benzoquinol methyltransferase activity

Cellular components

• extrinsic component of mitochondrial inner membrane
• mitochondrial inner membrane
• mitochondrial matrix
• mitochondrion
• protein-containing complex
• ubiquinone biosynthesis complex

Protein domainsUniProt · Pfam · InterPro

• UbiE/COQ5 methyltransferase, conserved site
• S-adenosyl-L-methionine-dependent methyltransferase superfamily
• UbiE/COQ5 methyltransferase
• ubiE/COQ5 methyltransferase family

KeywordsUniProt

• Membrane
• Methyltransferase
• Mitochondrion
• Mitochondrion inner membrane
• Primary mitochondrial disease
• S-adenosyl-L-methionine
• Transferase
• Transit peptide
• Ubiquinone biosynthesis

InteractionsUniProt · HPA

Protein binding partners of COQ5 in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• PYURF
• COQ4
• COQ6
• COQ9
• MFSD4A