Accumulation of lysosulfatide (sulfogalactosylsphingosine) in tissues of a boy with metachromatic leukodystrophy.

Abstract

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.

• Authors: K Toda, T Kobayashi, I Goto, T Kurokawa, K Ogomori
• Journal: Biochemical and biophysical research communications (Biochem Biophys Res Commun) Vol. 159 Issue 2 Pg. 605-11 (Mar 15 1989) ISSN: 0006-291X [Print] United States
• PMID: 2539117 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Psychosine
• psychosine-3'-sulfate ester
• Electron Transport Complex IV
• Sphingosine

Topics

• Animals
• Brain (metabolism)
• Child
• Electron Transport Complex IV (antagonists & inhibitors)
• Humans
• Leukodystrophy, Metachromatic (metabolism)
• Liver (enzymology)
• Male
• Mice
• Psychosine (analogs & derivatives, metabolism, pharmacology)
• Sciatic Nerve (metabolism)
• Sphingosine (analogs & derivatives)
• Spinal Cord (metabolism)