Disorders of Sphingolipid Metabolism

Myelin

“…Subsequent studies by Austin et al (1970) showed that whereas the cerebroside sulfotransferase activity was low in the brain, it was not consistently low in the kidney. It is probable that the low activity of the synthetic enzyme is a consequence of the loss of oligodendroglia in the bram (K. Suzuki and K. Suzuki, 1973a).…”

Myelin

“…Detailed reviews of the clinical, pathological, and chemical aspects of metachromatic leukodystrophy have been published Austin, 1973;Suzuki and Suzuki, 1973a), so only certain critical articles and those related specifically to myelin will be cited here. This is an autosomal recessive hereditary disease characterized by intracellular and extracellular accumulation of abnormal granules which stain yellow-brown (i.e., metachromatically) with acidic cresyl violet.…”

“…Subsequent studies by , showed that whereas the cerebroside sulfotransferase activity was low in the brain it was not consistently low in the kidney. It is probable that this abnormality is a consequence of the loss of oligodendroglia in the brain (Suzuki and Suzuki, 1973a).…”