Gene/Proteome Database (LMPD)

LMPD ID
LMP004889
Gene ID
57017
Species
Homo sapiens (Human)
Gene Name
coenzyme Q9
Gene Symbol
COQ9
Synonyms
C16orf49; COQ10D5
Alternate Names
ubiquinone biosynthesis protein COQ9, mitochondrial; coenzyme Q9 homolog
Chromosome
16
Map Location
16q21
Summary
This locus represents a mitochondrial ubiquinone biosynthesis gene. The encoded protein is likely necessary for biosynthesis of coenzyme Q10, as mutations at this locus have been associated with autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency.[provided by RefSeq, Sep 2010]
Orthologs
View orthologs and multiple alignments for COQ9

Proteins

ubiquinone biosynthesis protein COQ9, mitochondrial precursor
Refseq ID NP_064708
Protein GI 40789233
UniProt ID O75208
mRNA ID NM_020312
Length 318
RefSeq Status REVIEWED
MAAAAVSGALGRAGWRLLQLRCLPVARCRQALVPRAFHASAVGLRSSDEQKQQPPNSFSQQHSETQGAEKPDPESSHSPPRYTDQGGEEEEDYESEEQLQHRILTAALEFVPAHGWTAEAIAEGAQSLGLSSAAASMFGKDGSELILHFVTQCNTRLTRVLEEEQKLVQLGQAEKRKTDQFLRDAVETRLRMLIPYIEHWPRALSILMLPHNIPSSLSLLTSMVDDMWHYAGDQSTDFNWYTRRAMLAAIYNTTELVMMQDSSPDFEDTWRFLENRVNDAMNMGHTAKQVKSTGEALVQGLMGAAVTLKNLTGLNQRR
transit_peptide: 1..44 inference: protein motif:MitoProt II:1.101 note: The cleavage site of the transit peptide has not been experimentally determined. calculated_mol_wt: 4587 peptide sequence: MAAAAVSGALGRAGWRLLQLRCLPVARCRQALVPRAFHASAVGL

Gene Information

Entrez Gene ID
57017
Gene Name
coenzyme Q9
Gene Symbol
COQ9
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005739 IDA:UniProt C mitochondrion
GO:0006120 IEA:Ensembl P mitochondrial electron transport, NADH to ubiquinone
GO:0006744 IEA:UniProtKB-UniPathway P ubiquinone biosynthetic process

Domain Information

InterPro Annotations

Accession Description
IPR013718 COQ9
IPR012762 Ubiquinone biosynthesis protein COQ9

UniProt Annotations

Entry Information

Gene Name
coenzyme Q9
Protein Entry
COQ9_HUMAN
UniProt ID
O75208
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=O75208-1; Sequence=Displayed; Name=2; IsoId=O75208-2; Sequence=VSP_017683, VSP_017684; Note=No experimental confirmation available.;
Disease Coenzyme Q10 deficiency, primary, 5 (COQ10D5) [MIM
Function Involved in the biosynthesis of coenzyme Q.
Pathway Cofactor biosynthesis; ubiquinone biosynthesis.
Sequence Caution Sequence=AAF29004.1; Type=Frameshift; Positions=26, 133, 138, 141; Evidence= ;
Similarity Belongs to the COQ9 family.
Subcellular Location Mitochondrion .

Identical and Related Proteins

Unique RefSeq proteins for LMP004889 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
40789233 RefSeq NP_064708 318 ubiquinone biosynthesis protein COQ9, mitochondrial precursor

Identical Sequences to LMP004889 proteins

Reference Database Accession Length Protein Name
GI:40789233 GenBank ACE87678.1 318 coenzyme Q9 homolog (S. cerevisiae) protein [synthetic construct]
GI:40789233 GenBank ADQ32578.1 318 coenzyme Q9 homolog (S. cerevisiae), partial [synthetic construct]
GI:40789233 GenBank ADT48108.1 318 Sequence 125 from patent US 7842467
GI:40789233 GenBank ADT48112.1 318 Sequence 129 from patent US 7842467
GI:40789233 GenBank AHD78169.1 318 Sequence 25506 from patent US 8586006
GI:40789233 GenBank AIC51959.1 318 COQ9, partial [synthetic construct]

Related Sequences to LMP004889 proteins

Reference Database Accession Length Protein Name
GI:40789233 RefSeq XP_001146454.1 319 PREDICTED: ubiquinone biosynthesis protein COQ9, mitochondrial isoform X1 [Pan troglodytes]
GI:40789233 RefSeq XP_002761052.1 318 PREDICTED: ubiquinone biosynthesis protein COQ9, mitochondrial [Callithrix jacchus]
GI:40789233 RefSeq XP_003916991.1 319 PREDICTED: ubiquinone biosynthesis protein COQ9, mitochondrial isoform X4 [Papio anubis]
GI:40789233 RefSeq XP_007991627.1 318 PREDICTED: ubiquinone biosynthesis protein COQ9, mitochondrial isoform X2 [Chlorocebus sabaeus]
GI:40789233 RefSeq XP_010363783.1 319 PREDICTED: ubiquinone biosynthesis protein COQ9, mitochondrial isoform X2 [Rhinopithecus roxellana]
GI:40789233 RefSeq XP_010329640.1 318 PREDICTED: ubiquinone biosynthesis protein COQ9, mitochondrial [Saimiri boliviensis boliviensis]