Accumulation of lysosulfatide (sulfogalactosylsphingosine) in tissues of a boy with metachromatic leukodystrophy.
Abnormal accumulation of lysosulfatide (sulfogalactosylsphingosine) was evident in autopsied tissues from a boy with late-infantile metachromatic leukodystrophy. The concentration was high in the cerebral white matter, spinal cord and sciatic nerve (116-787 pmol/mg protein) and low in the cerebral gray matter, kidney and liver (4-40 pmol/mg protein). As is the case with galactosylsphingosine, lysosulfatide inhibited cytochrome c oxidase activity, in a dose-dependent manner. Judging from the tissue distribution of the accumulated lysosulfatide and because of the cytotoxicity, the lysosulfatide presumably explains the demyelination seen in the nervous tissues of patients with metachromatic leukodystrophy.
Toda
K
K
Department of Neurology, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Kobayashi
T
T
Goto
I
I
Kurokawa
T
T
Ogomori
K
K
D000818:Animals; D001921:Brain / Q000378:metabolism; D002648:Child; D003576:Electron Transport Complex IV / Q000037:antagonists & inhibitors; D006801:Humans; D007966:Leukodystrophy, Metachromatic / Q000378:metabolism*; D008099:Liver / Q000201:enzymology; D008297:Male; D051379:Mice; D011609:Psychosine / Q000031:analogs & derivatives* / Q000378:metabolism / Q000494:pharmacology; D012584:Sciatic Nerve / Q000378:metabolism; D013110:Sphingosine / Q000031:analogs & derivatives*; D013116:Spinal Cord / Q000378:metabolism
D011609:Psychosine; C019064:psychosine-3'-sulfate ester; D003576:Electron Transport Complex IV; D013110:Sphingosine