0000000000132189

AUTHOR

Fabien Gueugnon

showing 4 related works from this author

Dehydroepiandrosterone Induction of the Abcd2 and Abcd3 Genes encoding peroxisomal ABC Transporters

2003

Dehydroepiandrosterone (DHEA) is a peroxisome proliferator known to increase the expression of the genes encoding the peroxisomal s-oxidation enzymes in rodents. Using RT-PCR, we analysed the expression of the Abcd2 and Abcd3 genes encoding the peroxisomal ABC transporters ALDRP (ALD related protein) and PMP70 (70 kDa peroxisomal membrane protein) in primary cultures of rats hepatocytes treated with sulfated DHEA. We observed a time (12-72h) and dose (125-500μM) dependent increase in the expression of both genes.

chemistry.chemical_classificationSulfationEnzymeBiochemistrybiologychemistryABCD3ABCD2biology.proteinDehydroepiandrosteroneATP-binding cassette transporterPeroxisomeGene
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A novel cell model to study the function of the adrenoleukodystrophy-related protein

2006

X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder due to mutations in the ABCD1 (ALD) gene. ALDRP, the closest homolog of ALDP, has been shown to have partial functional redundancy with ALDP and, when overexpressed, can compensate for the loss-of-function of ALDP. In order to characterize the function of ALDRP and to understand the phenomenon of gene redundancy, we have developed a novel system that allows the controlled expression of the ALDRP-EGFP fusion protein (normal or non-functional mutated ALDRP) using the Tet-On system in H4IIEC3 rat hepatoma cells. The generated stable cell lines express negligible levels of endogenous ALDRP and doxycycline dosage-dependent lev…

Carcinoma Hepatocellularendocrine system diseasesRecombinant Fusion ProteinsBiophysicsGene redundancyATP-binding cassette transporterContext (language use)BiologyATP Binding Cassette Transporter Subfamily DProtein EngineeringTransfectionBiochemistryCell Line TumormedicineAnimalsAdrenoleukodystrophyMolecular BiologyGeneCell BiologyPeroxisomemedicine.diseaseFusion proteinRatsCell biologyDisease Models AnimalBiochemistryATP-Binding Cassette TransportersAdrenoleukodystrophyFunction (biology)
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Dehydroepiandrosterone up-regulates the Adrenoleukodystrophy-related gene (ABCD2) independently of PPAR alpha in rodents

2007

International audience; X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disease caused by mutations in the ABCD1 gene, which encodes a peroxisomal ABC transporter, ALDP, supposed to participate in the transport of very long chain fatty acids (VLCFA). The adrenoleukodystrophyrelated protein (ALDRP), which is encoded by the ABCD2 gene, is the closest homolog of ALDP and is considered as a potential therapeutic target since functional redundancy has been demonstrated between the two proteins. Pharmacological induction of Abcd2 by fibrates through the activation of PPARa has been demonstrated in rodent liver. DHEA, the most abundant steroid in human, is described as a PPARa activat…

MalePEROXISOMEProhormonePeroxisome proliferator-activated receptorATP-binding cassette transporterBiochemistryMice0302 clinical medicineABC TRANSPORTERSPPAR-ALPHAAdrenal GlandsTestisDHEACells Culturedchemistry.chemical_classification0303 health sciencesSex CharacteristicsbiologyBrainGeneral MedicineOrgan SizePeroxisome3. Good healthUp-RegulationLiverAdrenoleukodystrophyFemalemedicine.drugAndrostenediolmedicine.medical_specialtyADRENOLEUKODYSTROPHYATP Binding Cassette Transporter Subfamily D03 medical and health sciencesABCD3Internal medicinemedicineABCD2AnimalsPPAR alpha[SDV.BBM]Life Sciences [q-bio]/Biochemistry Molecular BiologyRats Wistar030304 developmental biologyActivator (genetics)Body Weightnutritional and metabolic diseasesMembrane ProteinsDehydroepiandrosteronemedicine.diseaseRatsMice Inbred C57BLEndocrinologychemistrybiology.proteinHepatocytesATP-Binding Cassette TransportersAcyl-CoA Oxidase030217 neurology & neurosurgery
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Pharmacological Induction of Redundant Genes for a Therapy of X-ALD

2003

X-linked adrenoleukodystrophy (X-ALD) is a recessive neurologic disease with an incidence among males of 1/17 000. Since the identification of the X-ALD gene (ABCD1) ten years ago (Mosser et al 1993), no satisfactory therapy has been available. A close homologue (ABCD2) was then cloned and presented as a putative modifier gene that could account for some of the extreme phenotypic variability of X-ALD (Lombard-Platet et al 1996). The inducibility of Abcd2 by the hypolipidemic drug fenofibrate in the liver of rodents (Albet et al 1997), correlated to a partial normalisation of the biochemical phenotype of X-ALD (Netik et al 1999), opened up the way of a pharmacological therapy of X-ALD. The b…

Pristanic acidcongenital hereditary and neonatal diseases and abnormalitiesFenofibrateendocrine system diseasesPharmacological therapybiologyPharmacologymedicine.diseasePhenylbutyrateBiochemical phenotypechemistry.chemical_compoundchemistrymedicineABCD2biology.proteinAdrenoleukodystrophyGenemedicine.drug
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