Molecular Target Synopsis
Overview
Domains and Structures
Drugs and Clinical Candidates
Druggability
Chemistry
Ligand Efficiency Plot
Pathways
Family Cladogram
Interaction Network
Gene Expression
Gene Copy Number Variation
RNAi
Mutations
Germline Genetics

COQ8A (Q8NI60) - Overview - Molecular Target Synopsis

Protein


COQ8A, Atypical kinase COQ8A, mitochondrial
Enzyme Classification 2.7.-.-
UniProt Q8NI60

Also Known as COQ8A_HUMAN, COQ8A, ADCK3, CABC1

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). Homodimer; homodimerizes via its transmembrane region (PubMed:25216398). Interacts with the multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9 (PubMed:27499294, PubMed:27499296).

5I35
STRUCTURE OF THE HUMAN MITOCHONDRIAL KINASE COQ8A R611K WITH AMPPNP (CEREBELLAR ATAXIA AND UBIQUINONE DEFICIENCY THROUGH LOSS OF UNORTHODOX KINASE ACTIVITY)
RCSB/PDB
Inspect Structure
See all 3D Structures for COQ8A

Isoforms / Transcripts (Protein Coding)


Sub-cellular localization


UniProt: COQ8A is active in the following subcellular-locations: membrane, mitochondrion.
GO terms: COQ8A is active in the following subcellular-locations: extrinsic component of mitochondrial inner membrane, integral component of membrane, mitochondrion.



UniProt
GO terms

Gene Copy Number Variation


In COSMIC - Cell Lines Project COQ8A has gain in 3 cell-lines, loss in 0 cell-lines and no signal in 1001 cell-lines. (see details)

Gene Expression


In NCI60, the highest expressing cell lines are:

In Array Express (RNA-seq of 675 commonly used human cancer cell lines), the highest expressing cell lines are:

In Array Express (RNA-seq of long poly adenylated RNA and long non poly adenylated RNA from ENCODE cell lines), the highest expressing cell lines are:

(see details)

3D Structures


For COQ8A there are:
2 structures (2 chains) solved
1 are solved in complex with at least one small molecule ligand



(see details)
Molecular Target 3D Synopsis

Screening and Chemistry


COQ8A has been screened with 305 compounds (406 bioactivities), 5 compounds have bioactivities that show binding affinity of <= 500nM (6 bioactivities). (see details)