NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES

BRAIN GLYCOSAMINOGLYCANS IN NORMALS AND FOUR TYPES OF MUCOPOLYSACCHARIDOSES

G. Constantopoulos, Rodney D McComb, A. S. Dekaban

Research output: Contribution to journalArticle

33 Citations (Scopus)

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 - Jan 1 1976
Externally publishedYes

Fingerprint

Mucopolysaccharidoses
Neurochemistry
Glycosaminoglycans
Brain
Dermatan Sulfate
Mucopolysaccharidosis III
Mucopolysaccharidosis II
Heparitin Sulfate
Mucopolysaccharidosis I
Molecular weight distribution
Liver
Intelligence
Spleen
Molecular Weight
Chemical analysis

ASJC Scopus subject areas

  • Biochemistry
  • Cellular and Molecular Neuroscience

Cite this

NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES : BRAIN GLYCOSAMINOGLYCANS IN NORMALS AND FOUR TYPES OF MUCOPOLYSACCHARIDOSES. / Constantopoulos, G.; McComb, Rodney D; Dekaban, A. S.

In: Journal of Neurochemistry, Vol. 26, No. 5, 01.01.1976, p. 901-908.

Research output: Contribution to journalArticle

@article{442f6e8a7cd84388bf716724ab38e24b,
title = "NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES: BRAIN GLYCOSAMINOGLYCANS IN NORMALS AND FOUR TYPES OF MUCOPOLYSACCHARIDOSES",
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",
author = "G. Constantopoulos and McComb, {Rodney D} and Dekaban, {A. S.}",
year = "1976",
month = "1",
day = "1",
doi = "10.1111/j.1471-4159.1976.tb06471.x",
language = "English (US)",
volume = "26",
pages = "901--908",
journal = "Journal of Neurochemistry",
issn = "0022-3042",
publisher = "Wiley-Blackwell",
number = "5",

}

TY - JOUR

T1 - NEUROCHEMISTRY OF THE MUCOPOLYSACCHARIDOSES

T2 - BRAIN GLYCOSAMINOGLYCANS IN NORMALS AND FOUR TYPES OF MUCOPOLYSACCHARIDOSES

AU - Constantopoulos, G.

AU - McComb, Rodney D

AU - Dekaban, A. S.

PY - 1976/1/1

Y1 - 1976/1/1

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

UR - http://www.scopus.com/inward/record.url?scp=0017273728&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0017273728&partnerID=8YFLogxK

U2 - 10.1111/j.1471-4159.1976.tb06471.x

DO - 10.1111/j.1471-4159.1976.tb06471.x

M3 - Article

VL - 26

SP - 901

EP - 908

JO - Journal of Neurochemistry

JF - Journal of Neurochemistry

SN - 0022-3042

IS - 5

ER -