NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES: BRAIN GLYCOSAMINOGLYCANS IN NORMALS AND FOUR TYPES OF MUCOPOLYSACCHARIDOSES

G. Constantopoulos, R. D. Mccomb, A. S. Dekaban

Research output: Contribution to journalArticle

34 Scopus citations

Abstract

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

Original languageEnglish (US)
Pages (from-to)901-908
Number of pages8
JournalJournal of Neurochemistry
Volume26
Issue number5
DOIs
StatePublished - May 1976

ASJC Scopus subject areas

  • Biochemistry
  • Cellular and Molecular Neuroscience

Fingerprint Dive into the research topics of 'NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES: BRAIN GLYCOSAMINOGLYCANS IN NORMALS AND FOUR TYPES OF MUCOPOLYSACCHARIDOSES'. Together they form a unique fingerprint.

  • Cite this