Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases.

Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases. Research Abstract Details 

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Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases. Abstract Text:

A Oohira,F Matsui,H Nogami,

In order to investigate the involvement of cartilage proteoglycans in the pathogenesis of human congenital skeletal disorders, proteoglycans were extracted with 4 M guanidine HCl from the iliac crest cartilage of children with various skeletal diseases; lysosomal storage diseases (group I), osteochondrodysplasias (group II) and controls (group III). The cartilage-type proteoglycan (PG-H) was purified and its chondroitin sulfate moiety was analyzed by digestion with chondroitinase-ABC. In group II and group III, the relative amounts of the unsaturated disaccharide products changed in an age-related manner; decrease (from 50% to 30%) of delta Di-4S with a compensatory increase (from 40% to 60%) of delta Di-6S with increasing age from 0 to 15 years. On the other hand, some cases in group I showed aberrant composition of the disaccharide products; a lower content of delta Di-4S with a correspondingly higher content of delta Di-6S. Patients in group I have clinically similar skeletal disorders, and the extent of the compositional abnormality seems to reflect the severity of the skeletal disorder. Therefore, one may consider that the aberrant composition of the glycosaminoglycans in PG-H is involved in the pathogenesis of the skeletal disorder of lysosomal storage diseases.

Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases. Publishing Authors By Initials

A Oohira,F Matsui,H Nogami,

For similar carbohydrates: polysaccharides: proteoglycans research abstracts see: carbohydrates: polysaccharides: proteoglycans research

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Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Journal of biochemistry

VOLUME: 99

Page Numbers: 1371-6

Journal Abbreviation: J. Biochem.

ISSN: 0021-924X

DAY: 19

MONTH: May

YEAR: 1986

Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases. Information

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LANGUAGE: eng

NlmUniqueID: 376600

Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases. Keywords Mesh Terms:

KEYWORDS: Proteoglycans

MESH TERMS: isolation & purification

Chemical & Substance for Abstract: Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases. Information

Substance Name: Chondroitin Sulfates

Registry Number: 9007-28-7

Grant and Affiliation Information for Aberrant composition of chondroitin sulfates in the cartilage-type proteoglycan isolated from the iliac crest of patients with some lysosomal storage diseases.

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Country: JAPAN

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MEDLINETA: J Biochem

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