Metachromatic leucodystrophy: isolation and chemical analysis of metachromatic granules.

The abnormal, cytoplasmic metachromatic granules found in the brain of metachromatic leucodystrophy were isolated in high purity. They show metachromasia by von Hirsch-Peiffer's cresyl violet method. Electron microscopy revealed very slight contaminations by other components, such as mitochondria, myelin, and ribosomes. Chemical analysis of two separately obtained collections has shown that the molar ratio of cholesterol to galactolipids to phosphatides is 1 : 1 : 1. Most of the galactolipids are sulfatides.