TY - JOUR
T1 - NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES
T2 - BRAIN GLYCOSAMINOGLYCANS IN NORMALS AND FOUR TYPES OF MUCOPOLYSACCHARIDOSES
AU - Constantopoulos, G.
AU - Mccomb, R. D.
AU - Dekaban, A. S.
PY - 1976/5
Y1 - 1976/5
N2 - 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
AB - 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
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U2 - 10.1111/j.1471-4159.1976.tb06471.x
DO - 10.1111/j.1471-4159.1976.tb06471.x
M3 - Article
C2 - 131847
AN - SCOPUS:0017273728
SN - 0022-3042
VL - 26
SP - 901
EP - 908
JO - Journal of Neurochemistry
JF - Journal of Neurochemistry
IS - 5
ER -