http://purl.uniprot.org/citations/10551832 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/10551832 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/10551832 | http://www.w3.org/2000/01/rdf-schema#comment | "Mammalian peroxisomal proteins adrenoleukodystrophy protein (ALDP), adrenoleukodystrophy-related protein (ALDRP), and 70-kDa peroxisomal protein (PMP70) belong to the superfamily of ATP-binding cassette (ABC) transporters. Unlike many ABC transporters that are single functional proteins with two related halves, ALDP, ALDRP, and PMP70 have the structure of ABC half-transporters. The dysfunction of ALDP is responsible for X-linked adrenoleukodystrophy (X-ALD), a neurodegenerative disorder in which saturated very long-chain fatty acids accumulate because of their impaired peroxisomal beta-oxidation. No disease has so far been associated with mutations of adrenoleukodystrophy-related or PMP70 genes. It has been proposed that peroxisomal ABC transporters need to dimerize to exert import functions. Using the yeast two-hybrid system, we show that homo-as well as heterodimerization occur between the carboxyl-terminal halves of ALDP, ALDRP, and PMP70. Two X-ALD disease mutations located in the carboxyl-terminal half of ALDP affect both homo- and heterodimerization of ALDP. Co-immunoprecipitation demonstrated the homodimerization of ALDP, the heterodimerization of ALDP with PMP70 or ALDRP, and the heterodimerization of ALDRP with PMP70. These results provide the first evidence of both homo- and heterodimerization of mammalian ABC half-transporters and suggest that the loss of ALDP dimerization plays a role in X-ALD pathogenesis."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.274.46.32738"xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.org/dc/terms/identifier | "doi:10.1074/jbc.274.46.32738"xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Benarous R."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Benarous R."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Berteaux-Lecellier V."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Berteaux-Lecellier V."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Aubourg P."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Aubourg P."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Cartier N."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Cartier N."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Janvier K."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Janvier K."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Liu L.X."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/author | "Liu L.X."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/date | "1999"xsd:gYear |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/date | "1999"xsd:gYear |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/name | "J. Biol. Chem."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/name | "J. Biol. Chem."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/pages | "32738-32743"xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/pages | "32738-32743"xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/title | "Homo- and heterodimerization of peroxisomal ATP-binding cassette half-transporters."xsd:string |
http://purl.uniprot.org/citations/10551832 | http://purl.uniprot.org/core/title | "Homo- and heterodimerization of peroxisomal ATP-binding cassette half-transporters."xsd:string |