Article
An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia.
Cell stress & chaperones - 1 Nov 2010
Nundlall Seema, Rajpar M Helen, Bell Peter A, Clowes Christopher, Zeeff Leo A H, Gardner Benjamin, Thornton David J, Boot-Handford Raymond P, Briggs Michael D
Abstract excerpt
Multiple epiphyseal dysplasia (MED) can result from mutations in matrilin-3, a structural protein of the cartilage extracellular matrix. We have previously shown that in a mouse model of MED the tibia growth plates were normal at birth but developed a progressive dysplasia characterised by the intracellular retention of mutant matrilin-3 and abnormal chondrocyte morphology. By 3 weeks of age, mutant mice...
Topics
- Animals
- Apoptosis
- Cartilage
- Chondrocytes
- Disease Models, Animal
- Endoplasmic Reticulum
- Extracellular Matrix Proteins
- Gene Expression Profiling
- Matrilin Proteins
- Mice
- Mutation
- Nerve Growth Factors
- Osteochondrodysplasias