0000000000154331

AUTHOR

Sabrina Albet

showing 4 related works from this author

Fibrate induction of the adrenoleukodystrophy-related gene (ABCD2)

2001

X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disease due to a defect in the ABCD1 (ALD) gene. ABCD1, and the two close homologues ABCD2 (ALDR) and ABCD3 (PMP70), are genes encoding ATP-binding cassette half-transporters of the peroxisomal membrane. As overexpression of the ABCD2 or ABCD3 gene can reverse the biochemical phenotype of X-ALD (reduced beta-oxidation of very-long-chain fatty acids), pharmacological induction of these partially redundant genes may represent a therapeutic approach to X-ALD. We previously reported that the ABCD2 and ABCD3 genes could be strongly induced by fibrates, which are hypolipidaemic drugs and peroxisome-proliferators in rodents. We provide e…

MaleTranscription GeneticMolecular Sequence DataResponse elementReceptors Cytoplasmic and NuclearATP-binding cassette transporterATP Binding Cassette Transporter Subfamily DBiochemistryMiceFenofibrateABCD3Sequence Homology Nucleic AcidABCD2medicineAnimalsHumansRats WistarAdrenoleukodystrophyPromoter Regions GeneticGeneHypolipidemic AgentsMice KnockoutBase SequencebiologyDNATransfectionPeroxisomemedicine.diseaseMolecular biologyRatsGene Expression Regulationbiology.proteinATP-Binding Cassette TransportersAdrenoleukodystrophyTranscription FactorsEuropean Journal of Biochemistry
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Copurification of dihydroxyacetone-phosphate acyl-transferase and other peroxisomal proteins from liver of fenofibrate-treated rats.

1997

Dihydroxyacetone-phosphate acyl-transferase (DHAP-AT), a peroxisomal membrane-bound enzyme that catalyzes the first step of ether-glycerolipid synthesis, was purified from liver of rats treated with fenofibrate, a peroxisome proliferator. The protocol first included isolation of peroxisomes, their purification through a discontinuous gradient and solubilization of membranes in CHAPS. DHAP-AT was further purified by four chromatographic steps, namely low-pressure size-exclusion, cation-exchange, hydroxylapatite and chromatofocusing. The chromatofocusing step led to a 4000-fold increase in the specific activity of DHAP-AT with respect to the liver homogenate with a yield of about 0.2%. Trypsi…

MaleMolecular Sequence DataBiochemistryMicrobodiesCopurificationchemistry.chemical_compoundFenofibrateProtein purificationAnimalsAmino Acid SequenceRats WistarPeptide sequenceDihydroxyacetone phosphatechemistry.chemical_classificationOxidase testChromatofocusingMembrane ProteinsGeneral MedicinePeroxisomeMolecular biologyRatsEnzymechemistryBiochemistryLiverSolubilitySequence AnalysisAcyltransferasesBiochimie
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The four murine peroxisomal ABC-transporter genes differ in constitutive, inducible and developmental expression.

1999

Four ATP-binding cassette (ABC) half-transporters have been identified in mammalian peroxisomes: adrenoleukodystrophy protein (ALDP), adrenoleukodystrophy-related protein (ALDRP), 70-kDa peroxisomal membrane protein (PMP70) and PMP70-related protein (P70R). Inherited defects in ALDP cause the neurodegenerative disorder X-linked adrenoleukodystrophy (X-ALD). By comparative Northern blot analyses we found each of the four murine peroxisomal ABC transporter mRNA species at maximum abundance only in a few tissues, which differed for each family member. The four genes were also regulated differentially during mouse brain development: ALDP mRNA was most abundant in embryonic brain and gradually d…

Response elementMolecular Sequence DataATP-binding cassette transporterMice Inbred StrainsBiologyATP Binding Cassette Transporter Subfamily DBiochemistryATP Binding Cassette Transporter Subfamily D Member 1MiceFenofibrateGene expressionmedicinePeroxisomesAnimalsNorthern blotATP Binding Cassette Transporter Subfamily B Member 1RNA MessengerPromoter Regions GeneticGeneHypolipidemic AgentsMice KnockoutMessenger RNABrainGene Expression Regulation DevelopmentalMembrane ProteinsProteinsBiological Transportmedicine.diseaseMolecular biologyNuclear receptorLiverAdrenoleukodystrophyATP-Binding Cassette TransportersEuropean journal of biochemistry
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Rat adrenoleukodystrophy-related (ALDR) gene: full-length cDNA sequence and new insight in expression.

2001

X-linked adrenoleukodystrophy (X-ALD) is an inherited demyelinating disorder due to mutations in the ALD gene, which encodes a peroxisomal ABC half-transporter (ALDP). It has been suggested that ALDP assembles with ALDRP (adrenoleukodystrophy-related protein), a close homologous half-transporter, to form a functional heterodimer. For the first time full-length ALDRP cDNA (5.5 kb) was cloned, and 5' and 3' RACE analysis revealed that alternative usage of polyadenylation sites generates the two transcripts of 3.0 and 5.5 kb observed in the rat in Northern blot analysis. Southern blotting and chromosomal mapping demonstrated one ALDR locus in the rat genome. Characterisation of the 3' flanking…

DNA ComplementaryPolyadenylationMolecular Sequence DataBiophysicsLocus (genetics)BiologyATP Binding Cassette Transporter Subfamily DBiochemistryMiceFenofibrateStructural BiologyComplementary DNAGene expressionGeneticsmedicineAnimalsNorthern blotAmino Acid SequenceCloning MolecularRats WistarAdrenoleukodystrophyGene3' Untranslated RegionsSouthern blotGene LibraryGeneticsBase SequenceBrainChromosome MappingGene Expression Regulation DevelopmentalProteinsmedicine.diseaseMolecular biologyRatsProtein BiosynthesisAdrenoleukodystrophyATP-Binding Cassette Transporters5' Untranslated RegionsBiochimica et biophysica acta
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