Profiling and Imaging of Phospholipids in Brains of Abcd1-Deficient Mice

Lipids
Kotaro HamaKazuaki Yokoyama

Abstract

ABCD1 is a gene responsible for X-linked adrenoleukodystrophy (X-ALD), and is critical for the transport of very long-chain fatty acids (VLCFA) into peroxisomes and subsequent β-oxidation. VLCFA-containing lipids accumulate in X-ALD patients, although the effect of ABCD1-deficiency on each lipid species in the central nervous system has not been fully characterized. In this study, each phospholipid and lysophospholipid species in Abcd1-deficient mice brains were profiled by liquid chromatography-mass spectrometry. Among the phospholipid and lysophospholipid species that are significantly more enriched in Abcd1-deficient mice brains, VLCFA were present in 75, 15, 5, 4, and 1 species of phosphatidylcholine, phosphatidylethanolamine, sphingomyelin, lysophosphatidylcholine, and lysophosphatidylethanolamine, respectively. Most VLCFA were incorporated at the sn-1 position of phosphatidylcholine and phosphatidylethanolamine. Among the phospholipid species that are significantly less enriched in Abcd1-deficient mice brains, odd-numbered saturated or mono-unsaturated fatty acyl moieties are contained in all phosphatidylcholine species. In addition, a number of phosphatidylglycerol, phosphatidylinositol, and phosphatidylserine species co...Continue Reading

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Citations

Nov 6, 2018·The Journal of Comparative Neurology·Guang Xu, Jianjun Li
Dec 17, 2019·Rapid Communications in Mass Spectrometry : RCM·Yuko NakashimaMitsutoshi Setou
Mar 18, 2020·Frontiers in Neuroscience·Frédéric DariosGiovanni Stevanin
Jul 11, 2020·Cellular & Molecular Biology Letters·Ambroise WuTomasz Trombik
May 8, 2019·Biological & Pharmaceutical Bulletin·Tsuneo Imanaka
Sep 15, 2018·Mass Spectrometry·Yuko Nakashima, Mitsutoshi Setou
Aug 20, 2019·Journal of Molecular Biology·Joan Bestard-EscalasGwendolyn Barceló-Coblijn
Apr 18, 2020·Biochimica Et Biophysica Acta. Molecular Basis of Disease·Angélica María Sabogal-GuáquetaGloria Patricia Cardona-Gómez

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Adrenoleukodystrophy

Adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder, is an X-linked disorder caused by a defect in the metabolism of long chain fatty acids leading to demyelination, neurodegeneration, and death. Here is the latest research.