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Literature summary extracted from
- Biogenesis and function of peroxisomes in human disease with a focus on the ABC transporter (2019), Biol. Pharm. Bull., 42, 649-665.
Cloned(Commentary)
| EC Number | Cloned (Comment) | Organism |
|---|---|---|
| 7.6.2.4 | expressed in Pichia pastoris | Homo sapiens |
| 7.6.2.8 | expressed in Pichia pastoris | Homo sapiens |
Localization
| EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|---|
| 7.6.2.4 | peroxisome | - |
Homo sapiens | 5777 | - |
| 7.6.2.8 | lysosome | - |
Homo sapiens | 5764 | - |
Natural Substrates/ Products (Substrates)
| EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 7.6.2.4 | ATP + H2O + fatty acyl-CoA[side 1] | Homo sapiens | - |
ADP + phosphate + fatty acyl-CoA[side 2] | - |
? |  |
| 7.6.2.8 | ATP + H2O + vitamin B12-[cobalamin-binding protein][side 1] | Homo sapiens | - |
ADP + phosphate + vitamin B12[side 2] + [cobalamin-binding protein][side 1] | - |
? | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 7.6.2.4 | Homo sapiens | P33897 | - |
- |
| 7.6.2.4 | Homo sapiens | P28288 | - |
- |
| 7.6.2.4 | Homo sapiens | Q9UBJ2 | - |
- |
| 7.6.2.8 | Homo sapiens | O14678 | - |
- |
Source Tissue
| EC Number | Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|---|
| 7.6.2.4 | kidney | - |
Homo sapiens | - |
| 7.6.2.4 | liver | - |
Homo sapiens | - |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 7.6.2.4 | ATP + H2O + (25R)-dihydroxycholestanoyl-CoA[side 1] | - |
Homo sapiens | ADP + phosphate + (25R)-dihydroxycholestanoyl-CoA[side 2] | - |
? | |
| 7.6.2.4 | ATP + H2O + (25R)-trihydroxycholestanoyl-CoA[side 1] | - |
Homo sapiens | ADP + phosphate + (25R)-trihydroxycholestanoyl-CoA[side 2] | - |
? | |
| 7.6.2.4 | ATP + H2O + docosahexaenoyl-CoA[side 1] | isoform ABCD2 has an affinity for polyunsaturated fatty acids such as docosahexaenoyl-CoA | Homo sapiens | ADP + phosphate + docosahexaenoyl-CoA[side 2] | - |
? | |
| 7.6.2.4 | ATP + H2O + fatty acyl-CoA[side 1] | - |
Homo sapiens | ADP + phosphate + fatty acyl-CoA[side 2] | - |
? | |
| 7.6.2.4 | ATP + H2O + phytanoyl-CoA[side 1] | - |
Homo sapiens | ADP + phosphate + phytanoyl-CoA[side 2] | - |
? | |
| 7.6.2.4 | ATP + H2O + pristanoyl-CoA[side 1] | - |
Homo sapiens | ADP + phosphate + pristanoyl-CoA[side 2] | - |
? | |
| 7.6.2.4 | ATP + H2O + tetracosahexaenoyl-CoA[side 1] | isoform ABCD2 has an affinity for polyunsaturated fatty acids such as tetracosahexaenoyl-CoA | Homo sapiens | ADP + phosphate + tetracosahexaenoyl-CoA[side 2] | - |
? | |
| 7.6.2.4 | additional information | isoform ABCD3 also possesses acyl-CoA thioesterase activity | Homo sapiens | ? | - |
? | |
| 7.6.2.4 | additional information | isoform ABCD1 also possesses acyl-CoA thioesterase activity | Homo sapiens | ? | - |
? | |
| 7.6.2.4 | additional information | isoform ABCD1+2+3 also possesses acyl-CoA thioesterase activity | Homo sapiens | ? | - |
? | |
| 7.6.2.8 | ATP + H2O + vitamin B12-[cobalamin-binding protein][side 1] | - |
Homo sapiens | ADP + phosphate + vitamin B12[side 2] + [cobalamin-binding protein][side 1] | - |
? | |
Subunits
| EC Number | Subunits | Comment | Organism |
|---|---|---|---|
| 7.6.2.4 | homodimer | - |
Homo sapiens |
| 7.6.2.8 | homodimer | - |
Homo sapiens |
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 7.6.2.4 | 70-kDa peroxisomal membrane protein | - |
Homo sapiens |
| 7.6.2.4 | ABCD 3 | - |
Homo sapiens |
| 7.6.2.4 | ABCD1 | - |
Homo sapiens |
| 7.6.2.4 | ABCD2 | - |
Homo sapiens |
| 7.6.2.4 | adrenoleukodystrophy protein | - |
Homo sapiens |
| 7.6.2.4 | ALDP | - |
Homo sapiens |
| 7.6.2.4 | ALDP-related protein | - |
Homo sapiens |
| 7.6.2.4 | ALDRP | - |
Homo sapiens |
| 7.6.2.4 | PMP70 | - |
Homo sapiens |
| 7.6.2.8 | ABCD 4 | - |
Homo sapiens |
| 7.6.2.8 | P70R | - |
Homo sapiens |
| 7.6.2.8 | PMP70-related protein | - |
Homo sapiens |
General Information
| EC Number | General Information | Comment | Organism |
|---|---|---|---|
| 7.6.2.4 | malfunction | a patient with isoform ABCD3 defect exhibits hepatosplenomegaly and severe liver disease | Homo sapiens |
| 7.6.2.4 | malfunction | the export of cholesterol from lysosomes is reduced by the mutation of isoform ABCD1. Mutation of isoform ABCD1 causes X-linked adrenoleukodystrophy | Homo sapiens |
| 7.6.2.4 | physiological function | isoform ABCD3 plays a role in the import of bile acid intermediates, as well as pristanic acid and phytanic acid | Homo sapiens |
| 7.6.2.4 | physiological function | isoform ABCD1 is involved in the transport of saturated and unsaturated very long-chain fatty acid-CoA into peroxisomes | Homo sapiens |
| 7.6.2.4 | physiological function | isoform ABCD2 is involved in the transport of saturated and unsaturated very long-chain fatty acid-CoA into peroxisomes. Isoform ABCD2 has a central role in the metabolism of monounsaturated and polyunsaturated rather than saturated very long-chain fatty acids, and may be involved in the regulation of oxidative stress and synthesis of docosahexaenoic acid | Homo sapiens |
| 7.6.2.8 | metabolism | isoform ABCD4 is involved in the transport of vitamin B12 from the lysosome to cytosol | Homo sapiens |
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