ATP-Binding and -Hydrolysis Activities of ALDP (ABCD1) and ALDRP (ABCD2), Human Peroxisomal ABC Proteins, Overexpressed in Sf21 Cells
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چکیده
منابع مشابه
Abcd2 Is a Strong Modifier of the Metabolic Impairments in Peritoneal Macrophages of Abcd1-Deficient Mice
The inherited peroxisomal disorder X-linked adrenoleukodystrophy (X-ALD), associated with neurodegeneration and inflammatory cerebral demyelination, is caused by mutations in the ABCD1 gene encoding the peroxisomal ATP-binding cassette (ABC) transporter ABCD1 (ALDP). ABCD1 transports CoA-esters of very long-chain fatty acids (VLCFA) into peroxisomes for degradation by β-oxidation; thus, ABCD1 d...
متن کاملPharmacological treatment based on gene redundancy: A novel therapeutic approach for X-linked adrenoleukodystrophy
X-linked adrenoleukodystrophy (X-ALD; OMIM 300100), the most frequent peroxisomal disorder, presents as a severe neurodegenerative disease with widely varying clinical phenotypes. The biochemical characteristic of X-ALD is the accumulation of very long-chain fatty acids (VLCFA), which should normally be degraded by the peroxisomal b-oxidation pathway, in plasma and tissues of X-ALD patients. It...
متن کاملSuppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins.
X-Linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder characterized by reduced peroxisomal very long chain fatty acid (VLCFA) beta-oxidation. The X - ALD gene product (ALDP) is a peroxisomal transmembrane protein with an ATP binding cassette (ABC). ALDP and three other ABC proteins (PMP70, ALDR, P70R) localize to the peroxisomal membrane. The function of this family of peroxisom...
متن کاملAdrenoleukodystrophy-related protein can compensate functionally for adrenoleukodystrophy protein deficiency (X-ALD): implications for therapy.
Inherited defects in the peroxisomal ATP-binding cassette (ABC) transporter adrenoleukodystrophy protein (ALDP) lead to the lethal peroxisomal disorder X-linked adrenoleukodystrophy (X-ALD), for which no efficient treatment has been established so far. Three other peroxisomal ABC transporters currently are known: adrenoleukodystrophy-related protein (ALDRP), 70 kDa peroxisomal membrane protein ...
متن کاملABCD2 Is a Direct Target of β-Catenin and TCF-4: Implications for X-Linked Adrenoleukodystrophy Therapy
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder caused by mutations in the ABCD1 gene that encodes the peroxisomal ATP-binding cassette (ABC) transporter subfamily D member 1 protein (ABCD1), which is referred to as the adrenoleukodystrophy protein (ALDP). Induction of the ABCD2 gene, the closest homolog of ABCD1, has been mentioned as a possible therapeutic option for the defec...
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ژورنال
عنوان ژورنال: Biological and Pharmaceutical Bulletin
سال: 2006
ISSN: 0918-6158,1347-5215
DOI: 10.1248/bpb.29.1836