Search results for "Abcd1"
showing 3 items of 3 documents
Inactivation of peroxisomal ABC transporters, ABCD1 and ABCD2 in BV-2 microglial cells : Towards a better understanding of X-linked adrenoleukodystro…
2018
X-linked adrenoleukodystrophy (X-ALD) is a severe neurodegenerative disorder characterized by very-long-chain fatty acid (VLCFA) accumulation resulting from a peroxisomal β-oxidation defect. The disease is caused by mutations in the ABCD1 gene, which encodes for a peroxisomal half ABC transporter predicted, like its closest homologue ABCD2, to participate in the entry of VLCFA-CoA into the peroxisome, the unique site of their β-oxidation. Progress in understanding the physiopathogenesis of X-ALD suffers from the lack of appropriate cell and animal models. Since peroxisomal defects in microglia seem to be a key element of the onset of the disease, we generated four microglial cell lines unab…
Structure-function analysis of peroxisomal ATP-binding cassette transporters using chimeric dimers
2014
Background: Peroxisomal ABC transporters are predicted to function as homodimers in mammals. [br/] Results: ABCD1 interacts with ABCD2. Chimeric proteins mimicking full-length dimers represent novel tools for functional study. Artificial homodimers and heterodimers are functional. [br/] Conclusion: Interchangeability between ABCD1 and ABCD2 is confirmed, but PUFA transport depends on ABCD2. [br/] Significance: For the first time, heterodimers in mammals are proven to be functional.[br/] ABCD1 and ABCD2 are two closely related ATP-binding cassette half-transporters predicted to homodimerize and form peroxisomal importers for fatty acyl-CoAs. Available evidence has shown that ABCD1 and ABCD2 …
Study of the natural cactus extracts protective effects on oxidative stress and inflammation related to peroxisomal β-oxidation deficiencies
2023
The objective of my thesis work was to better understand the role of microglial cells in neuroinflammation initiated in several neurovegetative peroxisomal leukodystrophies and to explore the protective effects of substances from the cactus Opuntia ficus indica. To this end, we used as an in vitro model microglial cells deficient in ATP Binding Cassette D transporters (ABCD 1 and ABCD 2) or acyl-CoA oxidase 1 (ACOX1). These are two BV-2 murine cell lines, deficient in Abcd1/Abcd2-/- or deficient in Acox1-/-, obtained elsewhere by gene editing using the CRISPR/Cas9 methodology. Our results made it possible to: (i) characterize, by differential centrifugation and isopycnic ultracentrifugation…