[HTML][HTML] The fate of cartilage oligomeric matrix protein is determined by the cell type in the case of a novel mutation in pseudoachondroplasia
BK Maddox, DR Keene, LY Sakai… - Journal of Biological …, 1997 - ASBMB
We have identified a novel missense mutation in a pseudoachondroplasia (PSACH) patient
in one of the type III repeats of cartilage oligomeric matrix protein (COMP). Enlarged lamellar
rough endoplasmic reticulum vesicles were shown to contain accumulated COMP along
with type IX collagen, a cartilage-specific component. COMP was secreted and assembled
normally into the extracellular matrix of tendon, demonstrating that the accumulation of
COMP in chondrocytes was a cell-specific phenomenon. We believe that the intracellular …
in one of the type III repeats of cartilage oligomeric matrix protein (COMP). Enlarged lamellar
rough endoplasmic reticulum vesicles were shown to contain accumulated COMP along
with type IX collagen, a cartilage-specific component. COMP was secreted and assembled
normally into the extracellular matrix of tendon, demonstrating that the accumulation of
COMP in chondrocytes was a cell-specific phenomenon. We believe that the intracellular …