Cartilage of I-cell disease.

Abstract:

:Biochemical analysis and morphologic observation of the iliac crest cartilage from 8-year-old girl with I-cell disease were performed. Instead of hypertrophic change of chondrocytes in the zone of cartilage-bone junction, there were small atrophic cells having many inclusion bodies accumulated with lamellar materials. Another characteristic finding in this zone was numerous cellular debris in the cartilage matrix. Significantly, a large amount of unsulfated chondroitin (21%) occurred in the cartilage with I-cell disease, and compensatory decrease of chondroitin 4-sulfate (14%) was observed. The percentage of chondroitin and chondroitin 4-sulfate on the control averaged 8.5 and 43%, respectively. The sedimentation profile of the proteoglycans closely resembled that of the normal cartilage.

journal_name

Pediatr Res

journal_title

Pediatric research

authors

Nogami H,Oohira A,Suzuki F,Tsuda K

doi

10.1203/00006450-198104000-00008

subject

Has Abstract

pub_date

1981-04-01 00:00:00

pages

330-4

issue

4 Pt 1

eissn

0031-3998

issn

1530-0447

journal_volume

15

pub_type

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