LIPID MAPS

LMPD ID

LMP012357

Gene ID

51117

Species

Homo sapiens (Human)

Gene Name

coenzyme Q4

Gene Symbol

COQ4

Synonyms

CGI-92;

Chromosome

9

Map Location

9q34.11

Summary

Coenzyme Q (CoQ) is a small lipophilic molecule that transports electrons between mitochondrial respiratory chain complexes and functions as a cofactor for mitochondrial enzymes. COQ4 is an enzyme involved in CoQ biosynthesis (Casarin et al., 2008 [PubMed 18474229]).[supplied by OMIM, Aug 2009]

Orthologs

View orthologs and multiple alignments for COQ4

ubiquinone biosynthesis protein COQ4 homolog, mitochondrial precursor
Refseq ID	NP_057119
Protein GI	166795285
UniProt ID	Q9Y3A0
mRNA ID	NM_016035
Length	265
MATLLRPVLRRLCGLPGLQRPAAEMPLRARSDGAGPLYSHHLPTSPLQKALLAAGSAAMALYNPYRHDMVAVLGETTGHRTLKVLRDQMRRDPEGAQILQERPRISTSTLDLGKLQSLPEGSLGREYLRFLDVNRVSPDTRAPTRFVDDEELAYVIQRYREVHDMLHTLLGMPTNILGEIVVKWFEAVQTGLPMCILGAFFGPIRLGAQSLQVLVSELIPWAVQNGRRAPCVLNLYYERRWEQSLRALREELGITAPPMHVQGLA
transit_peptide: 1..30 experiment: experimental evidence, no additional details recorded note: Mitochondrion. {ECO:0000255\|HAMAP-Rule:MF_03111}; propagated from UniProtKB/Swiss-Prot (Q9Y3A0.3) calculated_mol_wt: 3357 peptide sequence: MATLLRPVLRRLCGLPGLQRPAAEMPLRAR mat_peptide: 31..265 product: Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial experiment: experimental evidence, no additional details recorded note: propagated from UniProtKB/Swiss-Prot (Q9Y3A0.3) calculated_mol_wt: 26333 peptide sequence: SDGAGPLYSHHLPTSPLQKALLAAGSAAMALYNPYRHDMVAVLGETTGHRTLKVLRDQMRRDPEGAQILQERPRISTSTLDLGKLQSLPEGSLGREYLRFLDVNRVSPDTRAPTRFVDDEELAYVIQRYREVHDMLHTLLGMPTNILGEIVVKWFEAVQTGLPMCILGAFFGPIRLGAQSLQVLVSELIPWAVQNGRRAPCVLNLYYERRWEQSLRALREELGITAPPMHVQGLA

Entrez Gene ID

51117

Gene Name

coenzyme Q4

Gene Symbol

COQ4

Species

Homo sapiens

Gene Ontology (GO Annotations)

GO ID	Source	Type	Description
GO:0005743	IEA:UniProtKB-KW	C	mitochondrial inner membrane
GO:0005739	IDA:LIFEdb	C	mitochondrion
GO:0006744	IEA:UniProtKB-UniPathway	P	ubiquinone biosynthetic process

InterPro Annotations

Accession	Description
IPR007715	Ubiquinone biosynthesis protein Coq4
IPR027540	Ubiquinone biosynthesis protein Coq4, eukaryotes

Entry Information

Gene Name

coenzyme Q4

UniProt ID

Q9Y3A0

Species

Human

Comments

Comment Type	Description
Alternative Products	Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9Y3A0-1; Sequence=Displayed; Name=2; IsoId=Q9Y3A0-2; Sequence=VSP_036866;
Function	Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides. {ECO:0000255\|HAMAP- Rule:MF_03111, ECO:0000269\|PubMed:18474229}.
Pathway	Cofactor biosynthesis; ubiquinone biosynthesis
Similarity	Belongs to the COQ4 family. {ECO:0000255\|HAMAP- Rule:MF_03111}.
Subcellular Location	Isoform 1: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
Subunit	Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9
Tissue Specificity	Expressed ubiquitously, but at high levels in liver, lung and pancreas