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Literature summary for 7.6.2.4 extracted from
- X-linked adrenoleukodystrophy: very long-chain fatty acid metabolism, ABC half-transporters and the complicated route to treatment (2007), Mol. Genet. Metab., 90, 268-276.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | mutations in the gene encoding ALDP result in a devasting neurodegenerative disorder, X-linked adrenoleukodystrophy, X-ALD | Mammalia |
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
- |
Mammalia |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| peroxisomal membrane | - |
Mammalia | 5778 | - |
| peroxisomal membrane | - |
Saccharomyces cerevisiae | 5778 | - |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| ATP + H2O + fatty acyl CoA/cis | Mammalia | - |
ADP + phosphate + fatty acyl CoA/trans | - |
? |  |
| ATP + H2O + fatty acyl CoA/cis | Saccharomyces cerevisiae | - |
ADP + phosphate + fatty acyl CoA/trans | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Mammalia | - |
- |
- |
| Saccharomyces cerevisiae | - |
- |
- |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| adrenal gland | - |
Mammalia | - |
| blood | - |
Mammalia | - |
| blood plasma | - |
Mammalia | - |
| brain | - |
Mammalia | - |
| fibroblast | - |
Mammalia | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| ATP + H2O + fatty acyl CoA/cis | - |
Mammalia | ADP + phosphate + fatty acyl CoA/trans | - |
? | |
| ATP + H2O + fatty acyl CoA/cis | - |
Saccharomyces cerevisiae | ADP + phosphate + fatty acyl CoA/trans | - |
? | |
Subunits
| Subunits | Comment | Organism |
|---|---|---|
| dimer | homodimer or heterodimer | Mammalia |
| heterodimer | Pxa1, Pxa2 | Saccharomyces cerevisiae |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| ALDP | - |
Mammalia |
| ALDRP | - |
Mammalia |
| fatty-acyl-CoA-transporting ATPase | - |
Mammalia |
| fatty-acyl-CoA-transporting ATPase | - |
Saccharomyces cerevisiae |
| PMP69 | - |
Mammalia |
| PMP70 | - |
Mammalia |
| PMP70R | - |
Mammalia |
| Pxa | - |
Saccharomyces cerevisiae |
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Release 2023.1 (January 2023)
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