Reduced cell proliferation and increased apoptosis are significant pathological mechanisms in a murine model of mild pseudoachondroplasia resulting from a mutation in the C-terminal domain of COMP
about
Pseudoachondroplasia and multiple epiphyseal dysplasia: a 7-year comprehensive analysis of the known disease genes identify novel and recurrent mutations and provides an accurate assessment of their relative contributionA mouse model offers novel insights into the myopathy and tendinopathy often associated with pseudoachondroplasia and multiple epiphyseal dysplasiaEpigenetic regulation in chondrocyte phenotype maintenance for cell-based cartilage repairDefining the extracellular matrix using proteomicsMechanisms and models of endoplasmic reticulum stress in chondrodysplasiaThe crystal structure of the signature domain of cartilage oligomeric matrix protein: implications for collagen, glycosaminoglycan and integrin bindingLethal skeletal dysplasia in mice and humans lacking the golgin GMAP-210D469del-COMP retention in chondrocytes stimulates caspase-independent necroptosisCollagen XXVII organises the pericellular matrix in the growth plateAn unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia.A novel COMP mutation in a pseudoachondroplasia family of Chinese origin.Targeted induction of endoplasmic reticulum stress induces cartilage pathology.Transcriptional profiling of chondrodysplasia growth plate cartilage reveals adaptive ER-stress networks that allow survival but disrupt hypertrophyPseudoachondroplasia/COMP - translating from the bench to the bedside.Mild myopathy is associated with COMP but not MATN3 mutations in mouse models of genetic skeletal diseases.Abnormal chondrocyte apoptosis in the cartilage growth plate is influenced by genetic background and deletion of CHOP in a targeted mouse model of pseudoachondroplasiaIncreased classical endoplasmic reticulum stress is sufficient to reduce chondrocyte proliferation rate in the growth plate and decrease bone growth.The utility of mouse models to provide information regarding the pathomolecular mechanisms in human genetic skeletal diseases: The emerging role of endoplasmic reticulum stress (Review).F-spondin regulates chondrocyte terminal differentiation and endochondral bone formationChanges in the chondrocyte and extracellular matrix proteome during post-natal mouse cartilage development.A novel form of chondrocyte stress is triggered by a COMP mutation causing pseudoachondroplasia.Loss of matrilin 1 does not exacerbate the skeletal phenotype in a mouse model of multiple epiphyseal dysplasia caused by a Matn3 V194D mutationCollagen XII and XIV, new partners of cartilage oligomeric matrix protein in the skin extracellular matrix suprastructure.New therapeutic targets in rare genetic skeletal diseases.Alteration of proteoglycan sulfation affects bone growth and remodelingComparative proteomic analysis of normal and collagen IX null mouse cartilage reveals altered extracellular matrix composition and novel components of the collagen IX interactome.Ribozyme-mediated reduction of wild-type and mutant cartilage oligomeric matrix protein (COMP) mRNA and proteinCartilage homeostasis in health and rheumatic diseases.A novel transgenic mouse model of growth plate dysplasia reveals that decreased chondrocyte proliferation due to chronic ER stress is a key factor in reduced bone growth.The unfolded protein response and its relevance to connective tissue diseases.The chondrocytic journey in endochondral bone growth and skeletal dysplasia.Extracellular matrix and developing growth plate.An inducible cartilage oligomeric matrix protein mouse model recapitulates human pseudoachondroplasia phenotypeChop (Ddit3) is essential for D469del-COMP retention and cell death in chondrocytes in an inducible transgenic mouse model of pseudoachondroplasia.Armet/Manf and Creld2 are components of a specialized ER stress response provoked by inappropriate formation of disulphide bonds: implications for genetic skeletal diseases.Mutations targeting intermodular interfaces or calcium binding destabilize the thrombospondin-2 signature domain.Analysis of the cartilage proteome from three different mouse models of genetic skeletal diseases reveals common and discrete disease signatures.Genes uniquely expressed in human growth plate chondrocytes uncover a distinct regulatory network.Antisense Reduction of Mutant COMP Reduces Growth Plate Chondrocyte Pathology.Cartilage oligomeric matrix protein participates in the pathogenesis of liver fibrosis.
P2860
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P2860
Reduced cell proliferation and increased apoptosis are significant pathological mechanisms in a murine model of mild pseudoachondroplasia resulting from a mutation in the C-terminal domain of COMP
description
2007 nî lūn-bûn
@nan
2007 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
name
Reduced cell proliferation and ...... the C-terminal domain of COMP
@ast
Reduced cell proliferation and ...... the C-terminal domain of COMP
@en
Reduced cell proliferation and ...... the C-terminal domain of COMP
@nl
type
label
Reduced cell proliferation and ...... the C-terminal domain of COMP
@ast
Reduced cell proliferation and ...... the C-terminal domain of COMP
@en
Reduced cell proliferation and ...... the C-terminal domain of COMP
@nl
prefLabel
Reduced cell proliferation and ...... the C-terminal domain of COMP
@ast
Reduced cell proliferation and ...... the C-terminal domain of COMP
@en
Reduced cell proliferation and ...... the C-terminal domain of COMP
@nl
P2093
P2860
P50
P356
P1476
Reduced cell proliferation and ...... the C-terminal domain of COMP
@en
P2093
Dick Heinegård
Katarzyna A Piróg-Garcia
Lynette Knowles
Michael D Briggs
Roger S Meadows
P2860
P304
P356
10.1093/HMG/DDM155
P407
P577
2007-06-22T00:00:00Z