The biochemical defect of pseudoachondroplasia

Abstract

Histochemical, immunohistochemical, electron microscopical and microchemical studies were performed on growth cartilage biopsies in 4 cases of pseudoachondroplasia and in 4 controls. An accumulation of a non collagenous protein material into the rough endoplasmic reticulum of chondrocytes and the missing of a proteoglycan population of the cartilage were detected. The accumulated material was stained with antibodies against the core proteins of proteoglycans. In pseudoachondroplasia, an abnormal synthesized or processed protein core of a proteoglycan population is not properly transfered to the Golgi system.

DOI: 10.1007/BF00441206

2 Figures and Tables

Statistics

0200400600'87'90'93'96'99'02'05'08'11'14'17
Citations per Year

842 Citations

Semantic Scholar estimates that this publication has 842 citations based on the available data.

See our FAQ for additional information.

Cite this paper

@article{Stanescu1982TheBD, title={The biochemical defect of pseudoachondroplasia}, author={Victor Stanescu and Pierre Maroteaux and Relu Stanescu}, journal={European Journal of Pediatrics}, year={1982}, volume={138}, pages={221-225} }