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NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES: BRAIN GLYCOSAMINOGLYCANS IN NORMALS AND FOUR TYPES OF MUCOPOLYSACCHARIDOSES
Author(s) -
Constantopoulos G.,
Mccomb R. D.,
Dekaban A. S.
Publication year - 1976
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1976.tb06471.x
Subject(s) - glycosaminoglycan , dermatan sulfate , mucopolysaccharidosis type i , mucopolysaccharidosis , heparan sulfate , hurler syndrome , chemistry , mucopolysaccharidosis type ii , mucopolysaccharidosis i , disaccharide , endocrinology , medicine , biochemistry , disease , enzyme replacement therapy
— Glycosaminoglycan content, composition and molecular weight distribution were determined in cerebral gray and white matter, liver and spleen from normals and 7 patients with mucopolysaccharidosis; 4 were of Type I (Hurler), one Type II (Hunter), one Type IIIA (Sanfilippo A) and one Type V (Scheie). There was a 3 to 4‐fold increase in glycosaminoglycan content of the brains from patients with mucopolysaccharidosis Type I, II and IIIA, but only a 40% increase in the Type V patient. Partially degraded dermatan sulfate accounted for most of the increase in Types I, II and V. Highly fragmented heparan sulfate was the major glycosaminoglycan in the brain of the Type IIIA patient and was also a sizable component in Types I and II. Remarkably, the changes in the brain glycosaminoglycans of the Type V patient were minimal. He also was of normal intelligence