Astrocytes and mitochondria from adrenoleukodystrophy protein (ABCD1)-deficient mice reveal that the adrenoleukodystrophy-associated very long-chain fatty acids target several cellular energy-dependent functions

Article ID	Journal	Published Year	Pages	File Type
8259885	Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease	2015	12 Pages	PDF

Keywords

CALD HBSS FET FCS ΔΨm oligomycin AMN WST-1 X-linked adrenoleukodystrophy FCCP Rh123 X-ALD ABCD1 ALDP MDA Adrenoleukodystrophy protein carbonyl cyanide 4-trifluoromethoxyphenylhydrazone TBARS MBM VLCFA PTP CPA DMEM Hanks balanced salt solution Dulbecco's modified Eagle's medium ROS adrenomyeloneuropathy Astrocyte Peroxisomal disorder Cyclopiazonic acid very long chain fatty acids Permeability transition pore imm OLI RET inner mitochondrial membrane dihydroethidium Rhodamine 123 fetal calf serum malondialdehyde reverse electron transport thiobarbituric acid reactive substances Mitochondrion DHE Mitochondrial membrane potential Propidium iodide CRC Reactive oxygen species

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Astrocytes and mitochondria from adrenoleukodystrophy protein (ABCD1)-deficient mice reveal that the adrenoleukodystrophy-associated very long-chain fatty acids target several cellular energy-dependent functions

Authors

Nicol Kruska, Peter Schönfeld, Aurora Pujol, Georg Reiser,