Fig. 1
From: Peripheral neuropathy in metachromatic leukodystrophy: current status and future perspective
Sulfatide and lysosulfatide structures. Sulfatide (3-O-sulfogalactosylceramide) consists of a ceramide backbone (i.e. a long-chain base and a fatty acid chain) and a sulfated galactose moiety. Lysosulfatide (1-(3-O-sulfo-beta-D-galactosyl)sphingosine) is the deacylated form of sulfatide [2]. In MLD patients the lysosomal enzyme arylsulfatase A (ASA), which hydrolyzes the sulfate group in the degradation of sulfatide and lysosulfatide, is deficient, leading to accumulation of (lyso) sulfatides in various tissues (e.g. nervous tissue, gall bladder, kidneys and liver) [2]