Gene/Proteome Database (LMPD)
LMPD ID
LMP002411
Gene ID
Species
Homo sapiens (Human)
Gene Name
acyl-CoA oxidase 1, palmitoyl
Gene Symbol
Synonyms
ACOX; PALMCOX; SCOX
Alternate Names
peroxisomal acyl-coenzyme A oxidase 1; AOX; palmitoyl-CoA oxidase; straight-chain acyl-CoA oxidase; acyl-CoA oxidase, straight-chain; peroxisomal fatty acyl-CoA oxidase; acyl-Coenzyme A oxidase 1, palmitoyl
Chromosome
17
Map Location
17q25.1
EC Number
1.3.3.6
Summary
The protein encoded by this gene is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
| peroxisomal acyl-coenzyme A oxidase 1 isoform a | |
|---|---|
| Refseq ID | NP_004026 |
| Protein GI | 30089972 |
| UniProt ID | Q15067 |
| mRNA ID | NM_004035 |
| Length | 660 |
| RefSeq Status | REVIEWED |
| MNPDLRRERDSASFNPELLTHILDGSPEKTRRRREIENMILNDPDFQHEDLNFLTRSQRYEVAVRKSAIMVKKMREFGIADPDEIMWFKNFVHRGRPEPLDLHLGMFLPTLLHQATAEQQERFFMPAWNLEIIGTYAQTEMGHGTHLRGLETTATYDPETQEFILNSPTVTSIKWWPGGLGKTSNHAIVLAQLITKGKCYGLHAFIVPIREIGTHKPLPGITVGDIGPKFGYDEIDNGYLKMDNHRIPRENMLMKYAQVKPDGTYVKPLSNKLTYGTMVFVRSFLVGEAARALSKACTIAIRYSAVRHQSEIKPGEPEPQILDFQTQQYKLFPLLATAYAFQFVGAYMKETYHRINEGIGQGDLSELPELHALTAGLKAFTSWTANTGIEACRMACGGHGYSHCSGLPNIYVNFTPSCTFEGENTVMMLQTARFLMKSYDQVHSGKLVCGMVSYLNDLPSQRIQPQQVAVWPTMVDINSPESLTEAYKLRAARLVEIAAKNLQKEVIHRKSKEVAWNLTSVDLVRASEAHCHYVVVKLFSEKLLKIQDKAIQAVLRSLCLLYSLYGISQNAGDFLQGSIMTEPQITQVNQRVKELLTLIRSDAVALVDAFDFQDVTLGSVLGRYDGNVYENLFEWAKNSPLNKAEVHESYKHLKSLQSKL | |
| peroxisomal acyl-coenzyme A oxidase 1 isoform b | |
|---|---|
| Refseq ID | NP_009223 |
| Protein GI | 30089974 |
| UniProt ID | Q15067 |
| mRNA ID | NM_007292 |
| Length | 660 |
| RefSeq Status | REVIEWED |
| MNPDLRRERDSASFNPELLTHILDGSPEKTRRRREIENMILNDPDFQHEDLNFLTRSQRYEVAVRKSAIMVKKMREFGIADPDEIMWFKKLHLVNFVEPVGLNYSMFIPTLLNQGTTAQKEKWLLSSKGLQIIGTYAQTEMGHGTHLRGLETTATYDPETQEFILNSPTVTSIKWWPGGLGKTSNHAIVLAQLITKGKCYGLHAFIVPIREIGTHKPLPGITVGDIGPKFGYDEIDNGYLKMDNHRIPRENMLMKYAQVKPDGTYVKPLSNKLTYGTMVFVRSFLVGEAARALSKACTIAIRYSAVRHQSEIKPGEPEPQILDFQTQQYKLFPLLATAYAFQFVGAYMKETYHRINEGIGQGDLSELPELHALTAGLKAFTSWTANTGIEACRMACGGHGYSHCSGLPNIYVNFTPSCTFEGENTVMMLQTARFLMKSYDQVHSGKLVCGMVSYLNDLPSQRIQPQQVAVWPTMVDINSPESLTEAYKLRAARLVEIAAKNLQKEVIHRKSKEVAWNLTSVDLVRASEAHCHYVVVKLFSEKLLKIQDKAIQAVLRSLCLLYSLYGISQNAGDFLQGSIMTEPQITQVNQRVKELLTLIRSDAVALVDAFDFQDVTLGSVLGRYDGNVYENLFEWAKNSPLNKAEVHESYKHLKSLQSKL | |
| peroxisomal acyl-coenzyme A oxidase 1 isoform c | |
|---|---|
| Refseq ID | NP_001171968 |
| Protein GI | 297206874 |
| UniProt ID | Q15067 |
| mRNA ID | NM_001185039 |
| Length | 622 |
| RefSeq Status | REVIEWED |
| MILNDPDFQHEDLNFLTRSQRYEVAVRKSAIMVKKMREFGIADPDEIMWFKNFVHRGRPEPLDLHLGMFLPTLLHQATAEQQERFFMPAWNLEIIGTYAQTEMGHGTHLRGLETTATYDPETQEFILNSPTVTSIKWWPGGLGKTSNHAIVLAQLITKGKCYGLHAFIVPIREIGTHKPLPGITVGDIGPKFGYDEIDNGYLKMDNHRIPRENMLMKYAQVKPDGTYVKPLSNKLTYGTMVFVRSFLVGEAARALSKACTIAIRYSAVRHQSEIKPGEPEPQILDFQTQQYKLFPLLATAYAFQFVGAYMKETYHRINEGIGQGDLSELPELHALTAGLKAFTSWTANTGIEACRMACGGHGYSHCSGLPNIYVNFTPSCTFEGENTVMMLQTARFLMKSYDQVHSGKLVCGMVSYLNDLPSQRIQPQQVAVWPTMVDINSPESLTEAYKLRAARLVEIAAKNLQKEVIHRKSKEVAWNLTSVDLVRASEAHCHYVVVKLFSEKLLKIQDKAIQAVLRSLCLLYSLYGISQNAGDFLQGSIMTEPQITQVNQRVKELLTLIRSDAVALVDAFDFQDVTLGSVLGRYDGNVYENLFEWAKNSPLNKAEVHESYKHLKSLQSKL | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0016020 | IDA:UniProtKB | C | membrane |
| GO:0005739 | IEA:Ensembl | C | mitochondrion |
| GO:0005782 | TAS:Reactome | C | peroxisomal matrix |
| GO:0005778 | IEA:Ensembl | C | peroxisomal membrane |
| GO:0005777 | IDA:UniProtKB | C | peroxisome |
| GO:0003995 | IEA:InterPro | F | acyl-CoA dehydrogenase activity |
| GO:0003997 | IDA:UniProtKB | F | acyl-CoA oxidase activity |
| GO:0071949 | IDA:UniProtKB | F | FAD binding |
| GO:0005504 | IEA:Ensembl | F | fatty acid binding |
| GO:0050660 | IEA:InterPro | F | flavin adenine dinucleotide binding |
| GO:0016401 | IDA:UniProtKB | F | palmitoyl-CoA oxidase activity |
| GO:0030165 | IDA:MGI | F | PDZ domain binding |
| GO:0047485 | IPI:UniProtKB | F | protein N-terminus binding |
| GO:0005102 | IPI:UniProtKB | F | receptor binding |
| GO:0036109 | TAS:Reactome | P | alpha-linolenic acid metabolic process |
| GO:0044255 | TAS:Reactome | P | cellular lipid metabolic process |
| GO:0033540 | IMP:BHF-UCL | P | fatty acid beta-oxidation using acyl-CoA oxidase |
| GO:0019395 | IGI:UniProtKB | P | fatty acid oxidation |
| GO:0006091 | IMP:UniProtKB | P | generation of precursor metabolites and energy |
| GO:0055088 | IDA:UniProtKB | P | lipid homeostasis |
| GO:0006629 | IDA:UniProtKB | P | lipid metabolic process |
| GO:0016559 | IGI:UniProtKB | P | peroxisome fission |
| GO:2000189 | IGI:UniProtKB | P | positive regulation of cholesterol homeostasis |
| GO:0006693 | IMP:UniProtKB | P | prostaglandin metabolic process |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
| GO:0007283 | IEA:Ensembl | P | spermatogenesis |
| GO:0033559 | TAS:Reactome | P | unsaturated fatty acid metabolic process |
| GO:0000038 | IMP:BHF-UCL | P | very long-chain fatty acid metabolic process |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| hsa04024 | cAMP signaling pathway |
| hsa04146 | Peroxisome |
| hsa03320 | PPAR signaling pathway |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_121147 | alpha-linolenic acid (ALA) metabolism |
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
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative splicing; Named isoforms=3; Name=1; Synonyms=ACOX1a, SCOX-exon 3I; IsoId=Q15067-1; Sequence=Displayed; Name=2; Synonyms=ACOX1b, SCOX-exon 3II; IsoId=Q15067-2; Sequence=VSP_000146; Name=3; IsoId=Q15067-3; Sequence=VSP_046129, VSP_000146; Note=No experimental confirmation available.; |
| Biophysicochemical Properties | Kinetic parameters: KM=73 uM for palmitoyl-CoA (isoform 1) ; KM=90 uM for palmitoyl-CoA (isoform 2) ; pH dependence: Optimum pH is 8.5 for isoform 1 and 7.5-8.5 for isoform 2. ; Temperature dependence: Optimum temperature for isoform 1 at pH 7.5 is 40 degrees Celsius with no activity at 50 degrees Celsius. Optimum temperature for isoform 2 at pH 7.5 is 47.5 degrees Celsius with 57% activity retained at 50 degrees Celsius. ; |
| Catalytic Activity | Acyl-CoA + O(2) = trans-2,3-dehydroacyl-CoA + H(2)O(2). |
| Cofactor | Name=FAD; Xref=ChEBI |
| Disease | Adrenoleukodystrophy, pseudoneonatal (Pseudo-NALD) [MIM |
| 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. Isoform 2 is twice as active as isoform 1 against 16-hydroxy- palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl- CoA. |
| Miscellaneous | Isoform 1 and isoform 2 can reverse the Acox1 null phenotype in mouse which is characterized by severe microvesicular hepatic steatosis, sustained activation of Ppara, spontaneous massive peroxisome proliferation and eventual development of hepatocellular carcinomas. Isoform 2 is more effective in reversal of the phenotype than isoform 1 (PubMed:20195242). |
| Pathway | Lipid metabolism; peroxisomal fatty acid beta-oxidation. |
| Sequence Caution | Sequence=CAD97622.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence= ; |
| Similarity | Belongs to the acyl-CoA oxidase family. |
| Subcellular Location | Peroxisome. |
| Tissue Specificity | Widely expressed with highest levels of isoform 1 and isoform 2 detected in testis. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 levels are higher in brain, lung, muscle, white adipose tissue and testis. Levels are almost equal in heart. |
Identical and Related Proteins
Unique RefSeq proteins for LMP002411 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 30089972 | RefSeq | NP_004026 | 660 | peroxisomal acyl-coenzyme A oxidase 1 isoform a |
| 30089974 | RefSeq | NP_009223 | 660 | peroxisomal acyl-coenzyme A oxidase 1 isoform b |
| 297206874 | RefSeq | NP_001171968 | 622 | peroxisomal acyl-coenzyme A oxidase 1 isoform c |
Identical Sequences to LMP002411 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:30089974 | EMBL | CBU90611.1 | 660 | unnamed protein product [Homo sapiens] |
| GI:30089974 | EMBL | CBU96027.1 | 660 | unnamed protein product [Homo sapiens] |
| GI:30089974 | GenBank | ADT48184.1 | 660 | Sequence 201 from patent US 7842467 |
| GI:30089972 | GenBank | ADT48190.1 | 660 | Sequence 207 from patent US 7842467 |
| GI:30089974 | GenBank | AGX52704.1 | 660 | Sequence 5763 from patent US 8541208 |
| GI:30089974 | GenBank | AGX57641.1 | 660 | Sequence 15625 from patent US 8541208 |
| GI:30089974 | GenBank | AGX63425.1 | 660 | Sequence 26954 from patent US 8541208 |
Related Sequences to LMP002411 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:30089972 | DBBJ | BAF84482.1 | 660 | unnamed protein product [Homo sapiens] |
| GI:297206874 | DBBJ | BAF84482.1 | 660 | unnamed protein product [Homo sapiens] |
| GI:30089972 | GenBank | AAH08767.1 | 660 | Acyl-Coenzyme A oxidase 1, palmitoyl [Homo sapiens] |
| GI:30089974 | GenBank | EAW89349.1 | 660 | acyl-Coenzyme A oxidase 1, palmitoyl, isoform CRA_a [Homo sapiens] |
| GI:30089974 | GenBank | EAW89350.1 | 660 | acyl-Coenzyme A oxidase 1, palmitoyl, isoform CRA_a [Homo sapiens] |
| GI:30089972 | GenBank | EAW89351.1 | 660 | acyl-Coenzyme A oxidase 1, palmitoyl, isoform CRA_b [Homo sapiens] |
| GI:297206874 | GenBank | EAW89351.1 | 660 | acyl-Coenzyme A oxidase 1, palmitoyl, isoform CRA_b [Homo sapiens] |
| GI:297206874 | GenBank | EAW89352.1 | 660 | acyl-Coenzyme A oxidase 1, palmitoyl, isoform CRA_b [Homo sapiens] |
| GI:30089972 | GenBank | EAW89352.1 | 660 | acyl-Coenzyme A oxidase 1, palmitoyl, isoform CRA_b [Homo sapiens] |
| GI:30089972 | GenBank | ADT48185.1 | 660 | Sequence 202 from patent US 7842467 |
| GI:30089974 | GenBank | ADT48186.1 | 660 | Sequence 203 from patent US 7842467 |
| GI:30089974 | GenBank | ADT48187.1 | 660 | Sequence 204 from patent US 7842467 |
| GI:30089972 | GenBank | ADT48188.1 | 660 | Sequence 205 from patent US 7842467 |
| GI:297206874 | GenBank | ADT48188.1 | 660 | Sequence 205 from patent US 7842467 |
| GI:30089974 | GenBank | ADT48189.1 | 660 | Sequence 206 from patent US 7842467 |
| GI:297206874 | GenBank | ADT48190.1 | 660 | Sequence 207 from patent US 7842467 |
| GI:297206874 | RefSeq | NP_004026.2 | 660 | peroxisomal acyl-coenzyme A oxidase 1 isoform a [Homo sapiens] |
| GI:30089974 | RefSeq | XP_004041079.1 | 660 | PREDICTED: peroxisomal acyl-coenzyme A oxidase 1 [Gorilla gorilla gorilla] |