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Literature summary for 7.6.2.4 extracted from

  • Imanaka, T.
    Biogenesis and function of peroxisomes in human disease with a focus on the ABC transporter (2019), Biol. Pharm. Bull., 42, 649-665.
    View publication on PubMed
Search for more literature for 7.6.2.4

Cloned(Commentary)

Cloned (Comment) Organism
expressed in Pichia pastoris Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
peroxisome
-
 Homo sapiens 5777
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + H2O + fatty acyl-CoA[side 1] Homo sapiens
-
 ADP + phosphate + fatty acyl-CoA[side 2]
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens P33897
-
-
Homo sapiens P28288
-
-
Homo sapiens Q9UBJ2
-
-

Source Tissue

Source Tissue Comment Organism Textmining
kidney
-
 Homo sapiens
-
liver
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + H2O + (25R)-dihydroxycholestanoyl-CoA[side 1]
-
 Homo sapiens ADP + phosphate + (25R)-dihydroxycholestanoyl-CoA[side 2]
-
 ?
ATP + H2O + (25R)-trihydroxycholestanoyl-CoA[side 1]
-
 Homo sapiens ADP + phosphate + (25R)-trihydroxycholestanoyl-CoA[side 2]
-
 ?
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]
-
 ?
ATP + H2O + fatty acyl-CoA[side 1]
-
 Homo sapiens ADP + phosphate + fatty acyl-CoA[side 2]
-
 ?
ATP + H2O + phytanoyl-CoA[side 1]
-
 Homo sapiens ADP + phosphate + phytanoyl-CoA[side 2]
-
 ?
ATP + H2O + pristanoyl-CoA[side 1]
-
 Homo sapiens ADP + phosphate + pristanoyl-CoA[side 2]
-
 ?
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]
-
 ?
additional information isoform ABCD3 also possesses acyl-CoA thioesterase activity Homo sapiens ?
-
 ?
additional information isoform ABCD1 also possesses acyl-CoA thioesterase activity Homo sapiens ?
-
 ?
additional information isoform ABCD1+2+3 also possesses acyl-CoA thioesterase activity Homo sapiens ?
-
 ?

Subunits

Subunits Comment Organism
homodimer
-
 Homo sapiens

Synonyms

Synonyms Comment Organism
70-kDa peroxisomal membrane protein
-
 Homo sapiens
ABCD 3
-
 Homo sapiens
ABCD1
-
 Homo sapiens
ABCD2
-
 Homo sapiens
adrenoleukodystrophy protein
-
 Homo sapiens
ALDP
-
 Homo sapiens
ALDP-related protein
-
 Homo sapiens
ALDRP
-
 Homo sapiens
PMP70
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction a patient with isoform ABCD3 defect exhibits hepatosplenomegaly and severe liver disease Homo sapiens
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
physiological function isoform ABCD3 plays a role in the import of bile acid intermediates, as well as pristanic acid and phytanic acid Homo sapiens
physiological function isoform ABCD1 is involved in the transport of saturated and unsaturated very long-chain fatty acid-CoA into peroxisomes Homo sapiens
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