An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia

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 intra...

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Veröffentlicht in:	Cell stress & chaperones 2010-11, Vol.15 (6), p.835-849
Hauptverfasser:	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.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Apoptosis Biochemistry Biomedical and Life Sciences Biomedicine Cancer Research Cartilage Cartilage - pathology Cartilage - ultrastructure Cell Biology Cell growth Chondrocytes Chondrocytes - metabolism Disease Models, Animal Endoplasmic Reticulum - metabolism Epiphyses Extracellular Matrix Proteins - genetics Extracellular Matrix Proteins - metabolism Gene Expression Profiling Genetic mutation Immunology Matrilin Proteins Mice Mutation Nerve Growth Factors - genetics Nerve Growth Factors - metabolism Neurosciences Original Paper Osteochondrodysplasias - genetics Osteochondrodysplasias - metabolism Osteochondrodysplasias - pathology Phenotype Phenylbutyrates - pharmacology Ribs Ribs - cytology Secretion Tibia Time Factors Unfolded Protein Response Up-Regulation
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