Reduced brain cholesterol content in arylsulfatase A-deficient mice

Biochemical and Biophysical Research Communications
Dieter LütjohannMatthias Eckhardt

Abstract

Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by deficiency in arylsulfatase A (ASA). Concentrations of cholesterol and its metabolites were determined in ASA deficient [ASA(-/-)] mice which serve as an animal model of MLD. We observed a significant reduction in cholesterol content in the brain of adult ASA(-/-) mice when compared to wild-type controls. This was not due to loss of myelin, because ASA(-/-) mice do not demyelinate. Other cholesterol metabolites were not changed significantly in ASA(-/-) mice, except for an increase in lathosterol. Moreover, reduced cholesterol levels were also found in tissue samples from two juvenile MLD cases. Since high cholesterol levels are important for myelination, and various cellular processes, like vesicular trafficking and signal transduction, reduced cholesterol levels might be an important factor in the molecular pathology of MLD.

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Citations

Mar 10, 2007·Journal of Inherited Metabolic Disease·C SevinN Cartier
Jan 4, 2008·Journal of Molecular Neuroscience : MN·Olaf MaierWia Baron
May 10, 2008·Molecular Neurobiology·Matthias Eckhardt
Nov 24, 2007·Prostaglandins & Other Lipid Mediators·Raghavendra Pralhada Rao, Jairaj K Acharya
Nov 9, 2010·Journal of Neurochemistry·Alexander McCampbellMary Savage
Apr 21, 2018·Journal of Bioenergetics and Biomembranes·Valéria Sutti NunesEder Carlos Rocha Quintão

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