Skip Navigation

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Request Permissions
Google Scholar
Right arrow Articles by OOHIRA, A.
Right arrow Articles by NOGAMI, H.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by OOHIRA, A.
Right arrow Articles by NOGAMI, H.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

J. Biochem, 1986, Vol. 99, No. 5 1371-1376
© 1986 Japanese Biochemical Society

research-article

Aberrant Composition of Chondroitin Sulfates in the Cartilage-Type Proteoglycan Isolated from the Iliac Crest of Patients with Some Lysosomal Storage Diseases1

Atsuhiko OOHIRA*, Fumiko MATSUI* and Hiroshi NOGAMI**

*Department of Embryology Institute for Development Research Central Hospital, Kasugai, Aichi 480–03
**Department of Orthopaedic Surgery Central Hospital, Kasugai, Aichi 480–03

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.

1This work was supported in part by Grants-in-Aid for Scientific Research (Nos. 59570651 and 60114002) from the Ministry of Education, Science and Culture of Japan and Research Grant No. 59–296 from the Ishida Foundation.


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?




Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.