Achondrogenesis type II, abnormalities of extracellular matrix.

Abstract:

:Immune and lectin histochemical and microchemical methods were employed to study growth cartilage from seven cases of achondrogenesis type II (Langer-Saldino). The normal architecture of the epiphyseal and growth plate cartilage was replaced by a morphologically heterogeneous tissue. Some areas were comprised of vascular canals surrounded by extensive fibrous tissue and enlarged cells that had the appearance and histochemical characteristics of hypertrophic chondrocytes. Other areas contained a mixture of cells ranging from small to the enlarged chondrocytes. The extracellular matrix in the latter areas was more abundant and had characteristics of both precartilage mesenchymal matrix and typical cartilage matrix; it contained types I and II collagen, cartilage proteoglycan, fibronectin, and peanut agglutinin binding glycoconjugate(s). Peptide mapping of cyanogen bromide cartilage collagen peptides revealed the presence of types I and II collagen. These observations could be explained by a defect in the biosynthesis of type II collagen or in chondrocyte differentiation.

journal_name

Pediatr Res

journal_title

Pediatric research

authors

Horton WA,Machado MA,Chou JW,Campbell D

doi

10.1203/00006450-198709000-00017

subject

Has Abstract

pub_date

1987-09-01 00:00:00

pages

324-9

issue

3

eissn

0031-3998

issn

1530-0447

journal_volume

22

pub_type

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