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X-linked adrenoleukodystrophy (X-ALD) is a severe neurological disease characterized by progressive demyelination within the CNS, adrenal insufficiency, and is associated with an accumulation of saturated very long chain fatty acids in plasma and tissues of patients. iNKT cells, a distinct lineage of T cells recognizing glycolipid antigens through CD1d molecules, exert immunoregulatory functions and can prevent various immune mediated-pathologies. In ALD patients, but not in ALD deficient mice, iNKT cell frequency and CD1d expression on the surface of B cells are slightly decreased. However, such minor differences might not influence the pathogenesis of the disease.

Original publication

DOI

10.1016/j.jneuroim.2010.09.003

Type

Journal article

Journal

J Neuroimmunol

Publication Date

15/12/2010

Volume

229

Pages

204 - 211

Keywords

Adolescent, Adrenoleukodystrophy, Adult, Aged, Animals, Antigen-Presenting Cells, Antigens, CD1, Antigens, CD1d, Brefeldin A, Child, Cytokines, Disease Models, Animal, Flow Cytometry, Glycoproteins, Glycosphingolipids, Humans, Lipid Metabolism, Male, Mice, Mice, Inbred C57BL, Middle Aged, Natural Killer T-Cells, Protein Synthesis Inhibitors, Young Adult