Gene/Proteome Database (LMPD)

LMPD ID
LMP002274
Gene ID
11430
Species
Mus musculus (Mouse)
Gene Name
acyl-Coenzyme A oxidase 1, palmitoyl
Gene Symbol
Acox1
Synonyms
AOX; Acox; D130055E20Rik; Paox
Alternate Names
peroxisomal acyl-coenzyme A oxidase 1; palmitoyl-CoA oxidase; peroxisomal fatty acyl-coenzyme A oxidase; peroxisomal straight-chain Acyl-CoA oxidase
Chromosome
11
Map Location
11 E2|11
EC Number
1.3.3.6
Summary
This gene encodes a member of the acyl-coenzyme A oxidase family. The encoded protein is localized to peroxisomes and is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-coenzyme A to 2-trans-enoyl-coenzyme A. Disruption of this gene results in microvesicular steatohepatitis, spontaneous peroxisome proliferation, and the eventual development of hepatocellular carcinomas. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2012]
Orthologs
View orthologs and multiple alignments for Acox1

Proteins

peroxisomal acyl-coenzyme A oxidase 1 isoform 1
Refseq ID NP_056544
Protein GI 66793429
UniProt ID Q9R0H0
mRNA ID NM_015729
Length 661
RefSeq Status REVIEWED
MNPDLRKERAAATFNPELITHILDGSPENTRRRREIENLILNDPDFQHEDYNFLTRSQRYEVAVKKSATMVKKMREFGIADPEEIMWFKNSVHRGHPEPLDLHLGMFLPTLLHQATEEQQERFFMPAWNLEITGTYAQTEMGHGTHLRGLETTATYDPKTQEFILNSPTVTSIKWWPGGLGKTSNHAIVLAQLITRGECYGLHAFVVPIREIGTHKPLPGITVGDIGPKFGYEEMDNGYLKMDNYRIPRENMLMKYAQVKPDGTYVKPLSNKLTYGTMVFVRSFLVGSAAQSLSKACTIAIRYSAVRRQSEIKRSEPEPQILDFQTQQYKLFPLLATAYAFHFLGRYIKETYMRINESIGQGDLSELPELHALTAGLKAFTTWTANAGIEECRMACGGHGYSHSSGIPNIYVTFTPACTFEGENTVMMLQTARFLMKIYDQVQSGKLVGGMVSYLNDLPSQRIQPQQVAVWPTLVDINSLDSLTEAYKLRAARLVEIAAKNLQAQVSHRKSKEVAWNLTSVDLVRASEAHCHYVTVKVFADKLPKIQDRAVQAVLRNLCLLYSLYGISQKGGDFLEGNIITGAQMSQVNSRILELLTVTRPNAVALVDAFDFKDVTLGSVLGRYDGNVYENLFEWAKKSPLNKTEVHESYYKHLKPLQSKL
peroxisomal acyl-coenzyme A oxidase 1 isoform 2
Refseq ID NP_001258827
Protein GI 429484484
UniProt ID Q9R0H0
mRNA ID NM_001271898
Length 661
RefSeq Status REVIEWED
MNPDLRKERAAATFNPELITHILDGSPENTRRRREIENLILNDPDFQHEDYNFLTRSQRYEVAVKKSATMVKKMREFGIADPEEIMWFKKLHMVNFVEPVGLNYSMFIPTLLNQGTTAQQEKWMHPSQELQIIGTYAQTEMGHGTHLRGLETTATYDPKTQEFILNSPTVTSIKWWPGGLGKTSNHAIVLAQLITRGECYGLHAFVVPIREIGTHKPLPGITVGDIGPKFGYEEMDNGYLKMDNYRIPRENMLMKYAQVKPDGTYVKPLSNKLTYGTMVFVRSFLVGSAAQSLSKACTIAIRYSAVRRQSEIKRSEPEPQILDFQTQQYKLFPLLATAYAFHFLGRYIKETYMRINESIGQGDLSELPELHALTAGLKAFTTWTANAGIEECRMACGGHGYSHSSGIPNIYVTFTPACTFEGENTVMMLQTARFLMKIYDQVQSGKLVGGMVSYLNDLPSQRIQPQQVAVWPTLVDINSLDSLTEAYKLRAARLVEIAAKNLQAQVSHRKSKEVAWNLTSVDLVRASEAHCHYVTVKVFADKLPKIQDRAVQAVLRNLCLLYSLYGISQKGGDFLEGNIITGAQMSQVNSRILELLTVTRPNAVALVDAFDFKDVTLGSVLGRYDGNVYENLFEWAKKSPLNKTEVHESYYKHLKPLQSKL

Gene Information

Entrez Gene ID
11430
Gene Name
acyl-Coenzyme A oxidase 1, palmitoyl
Gene Symbol
Acox1
Species
Mus musculus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005737 IDA:MGI C cytoplasm
GO:0005739 IDA:MGI C mitochondrion
GO:0005778 IDA:MGI C peroxisomal membrane
GO:0005777 IDA:UniProtKB C peroxisome
GO:0071949 IEA:Ensembl F FAD binding
GO:0030165 ISO:MGI F PDZ domain binding
GO:0003995 IEA:InterPro F acyl-CoA dehydrogenase activity
GO:0003997 TAS:MGI F acyl-CoA oxidase activity
GO:0005504 IEA:Ensembl F fatty acid binding
GO:0016401 IEA:Ensembl F palmitoyl-CoA oxidase activity
GO:0006635 TAS:MGI P fatty acid beta-oxidation
GO:0033540 IEA:UniProtKB-UniPathway P fatty acid beta-oxidation using acyl-CoA oxidase
GO:0019395 IMP:UniProtKB P fatty acid oxidation
GO:0006091 IEA:Ensembl P generation of precursor metabolites and energy
GO:0055088 IEA:Ensembl P lipid homeostasis
GO:0016559 IEA:Ensembl P peroxisome fission
GO:2000189 IEA:Ensembl P positive regulation of cholesterol homeostasis
GO:0006693 IEA:Ensembl P prostaglandin metabolic process
GO:0007283 IMP:MGI P spermatogenesis
GO:0000038 IEA:Ensembl P very long-chain fatty acid metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
mmu03320 PPAR signaling pathway
mmu04146 Peroxisome
mmu04024 cAMP signaling pathway

Domain Information

InterPro Annotations

Accession Description
IPR009075 Acyl-CoA dehydrogenase/oxidase C-terminal
IPR013786 Acyl-CoA dehydrogenase/oxidase, N-terminal
IPR009100 Acyl-CoA dehydrogenase/oxidase, N-terminal and middle domain
IPR012258 Acyl-CoA oxidase
IPR002655 Acyl-CoA oxidase, C-terminal
IPR006091 Acyl-CoA oxidase/dehydrogenase, central domain
IPR029320 Acyl-coenzyme A oxidase, N-terminal

UniProt Annotations

Entry Information

Gene Name
acyl-Coenzyme A oxidase 1, palmitoyl
Protein Entry
ACOX1_MOUSE
UniProt ID
Q9R0H0
Species
Mouse

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9R0H0-1; Sequence=Displayed; Name=2; IsoId=Q9R0H0-2; Sequence=VSP_042477;
Catalytic Activity Acyl-CoA + O(2) = trans-2,3-dehydroacyl-CoA + H(2)O(2).
Cofactor Name=FAD; Xref=ChEBI:CHEBI:57692; Evidence={ECO:0000250};
Disruption Phenotype Severe microvesicular hepatic steatosis, sustained activation of Ppara, spontaneous massive peroxisome proliferation and eventual development of hepatocellular carcinomas. {ECO:0000269|PubMed:20195242}.
Function Catalyzes the desaturation of acyl-CoAs to 2-trans- enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs (By similarity). {ECO:0000250}.
Pathway Lipid metabolism; peroxisomal fatty acid beta-oxidation.
Sequence Caution Sequence=BAE41642.1; Type=Erroneous initiation; Note=Translation N-terminally extended.; Evidence={ECO:0000305};
Similarity Belongs to the acyl-CoA oxidase family. {ECO:0000305}.
Subcellular Location Peroxisome {ECO:0000250}.
Tissue Specificity Highest levels of isoform 1 are found in liver and kidney while highest levels of isoform 2 are found in white adipose tissue. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 is expressed at higher levels in brain, heart, lung, muscle, white adipose tissue and testis. {ECO:0000269|PubMed:20195242}.

Identical and Related Proteins

Unique RefSeq proteins for LMP002274 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
66793429 RefSeq NP_056544 661 peroxisomal acyl-coenzyme A oxidase 1 isoform 1
429484484 RefSeq NP_001258827 661 peroxisomal acyl-coenzyme A oxidase 1 isoform 2

Identical Sequences to LMP002274 proteins

Reference Database Accession Length Protein Name
GI:429484484 DBBJ BAA86870.1 661 peroxisomal acyl-CoA oxidase [Mus musculus]
GI:66793429 EMBL CBU85812.1 661 unnamed protein product [Mus musculus]
GI:66793429 EMBL CBU90593.1 661 unnamed protein product [Mus musculus]
GI:66793429 EMBL CBU96009.1 661 unnamed protein product [Mus musculus]
GI:429484484 GenBank EDL34562.1 661 acyl-Coenzyme A oxidase 1, palmitoyl, isoform CRA_a [Mus musculus]
GI:66793429 GenBank AGX52686.1 661 Sequence 5727 from patent US 8541208
GI:66793429 GenBank AGX57623.1 661 Sequence 15589 from patent US 8541208
GI:66793429 GenBank AGX63407.1 661 Sequence 26918 from patent US 8541208
GI:429484484 SwissProt Q9R0H0.5 661 RecName: Full=Peroxisomal acyl-coenzyme A oxidase 1; Short=AOX; AltName: Full=Palmitoyl-CoA oxidase [Mus musculus]

Related Sequences to LMP002274 proteins

Reference Database Accession Length Protein Name
GI:429484484 EMBL CBU90612.1 661 unnamed protein product [Mus musculus]
GI:429484484 EMBL CBU96028.1 661 unnamed protein product [Mus musculus]
GI:429484484 GenBank AAB62926.1 661 peroxisomal acyl-CoA oxidase [Mus musculus]
GI:429484484 GenBank AGX52705.1 661 Sequence 5765 from patent US 8541208
GI:429484484 GenBank AGX57642.1 661 Sequence 15627 from patent US 8541208
GI:429484484 GenBank AGX63426.1 661 Sequence 26956 from patent US 8541208
GI:66793429 PDB 1IS2 661 Chain A, Crystal Structure Of Peroxisomal Acyl-coa Oxidase-ii From Rat Liver
GI:66793429 PDB 1IS2 661 Chain B, Crystal Structure Of Peroxisomal Acyl-coa Oxidase-ii From Rat Liver
GI:66793429 PDB 2DDH 661 Chain A, Crystal Structure Of Acyl-Coa Oxidase Complexed With 3-Oh-Dodecanoate
GI:66793429 RefSeq XP_005069961.1 661 PREDICTED: peroxisomal acyl-coenzyme A oxidase 1 isoform X1 [Mesocricetus auratus]
GI:66793429 RefSeq XP_006993715.1 661 PREDICTED: peroxisomal acyl-coenzyme A oxidase 1 isoform X1 [Peromyscus maniculatus bairdii]
GI:66793429 SwissProt Q9R0H0.5 661 RecName: Full=Peroxisomal acyl-coenzyme A oxidase 1; Short=AOX; AltName: Full=Palmitoyl-CoA oxidase [Mus musculus]