Add time:08/12/2019         Source:sciencedirect.com

Publisher SummarySulfatide or ceramide galactosyl-3’-sulfate is a major sulfoglycolipid of the mammalian nervous system, kidney, and spleen and is present in low concentrations in other tissues. In the nervous system, sulfatide is an integral constituent of myelin membranes and is biosynthesized by oligodendroglial cells in the central nervous system and by Schwann cells in the peripheral nervous system. Extensive reviews on the chemistry, metabolism, and functional distribution of sulfoglycolipids, including sulfatide, have appeared. This chapter discusses methods of analysis of sulfatide by high-performance liquid chromatography (HPLC) and of the enzymes sulfatidase (EC 3.1.6.8) and sulfotransferase (EC 2.8.2.11) that are involved in its metabolism. The analysis of sulfatide and the characterization of sulfatidase have been studied intensively since the early 1970s because of the recognition that sulfatide accumulates in tissues, particularly brain, of patients with a lysosomal storage disorder, metachromatic leukodystrophy (MLD). Different clinical forms of MLD have been described and nearly all cases are because of the deficient enzyme activity of sulfatidase, more commonly known as “arylsulfatase A” (ASA). Mutations in the ASA gene, in turn, are responsible for this autosomal recessive disorder. Rare cases of ASA deficiency are because of a deficiency of an activator protein, saposin B that works in concert with ASA to hydrolyze the glycolipid substrate. This latter disorder, also inherited as an autosomal recessive condition, is because of mutations in the prosaposin gene. All forms of MLD are associated with a failure to adequately catabolize sulfatide with a consequent toxic accumulation of this glycolipid.

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