MANNOSIDOSIS - ISOLATION OF OLIGOSACCHARIDE STORAGE MATERIAL FROM BRAIN

Gray matter of the brain from a patient with a new storage disease was extracted with water. Gel filtration and anion and cation exchange chromatography were used to isolate the material stored. The material was found to have a molecular weight probably less than 5,000 and was heterogeneous on gel filtration. One of the fractions isolated was composed of mannose and glucosamine in the proportions 4.6:1. Only extremely small amounts of amino acids, galactosamine, and uronic acids were found. It is suggested that the new disease be termed mannosidosis, referring to the mannoserich oligosaccharide material which is stored. It is believed that this material derives from glycoproteins. © 1969 The C. V. Mosby Company.