Decreased chondrocyte proliferation and dysregulated apoptosis in the cartilage growth plate are key features of a murine model of epiphyseal dysplasia caused by a matn3 mutation.
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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 contributionEpigenetic regulation in chondrocyte phenotype maintenance for cell-based cartilage repairMechanisms and models of endoplasmic reticulum stress in chondrodysplasiaAxonemal positioning and orientation in three-dimensional space for primary cilia: what is known, what is assumed, and what needs clarificationDerlin-2-deficient mice reveal an essential role for protein dislocation in chondrocytesCollagen XXVII organises the pericellular matrix in the growth plateA structure of a collagen VI VWA domain displays N and C termini at opposite sides of the protein.An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia.Targeted induction of endoplasmic reticulum stress induces cartilage pathology.Mild myopathy is associated with COMP but not MATN3 mutations in mouse models of genetic skeletal diseases.Increased 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).Changes in the chondrocyte and extracellular matrix proteome during post-natal mouse cartilage development.Severe Extracellular Matrix Abnormalities and Chondrodysplasia in Mice Lacking Collagen Prolyl 4-Hydroxylase Isoenzyme II in Combination with a Reduced Amount of Isoenzyme I.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 mutationNew 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.Cartilage 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.Matrilin-2 is a widely distributed extracellular matrix protein and a potential biomarker in the early stage of osteoarthritis in articular cartilage.Arsenite-induced endoplasmic reticulum-dependent apoptosis through disturbance of calcium homeostasis in HBE cell line.Matrilin-3 chondrodysplasia mutations cause attenuated chondrogenesis, premature hypertrophy and aberrant response to TGF-β in chondroprogenitor cellsArmet/Manf and Creld2 are components of a specialized ER stress response provoked by inappropriate formation of disulphide bonds: implications for genetic skeletal diseases.Characterization of recombinantly expressed matrilin VWA domains.Analysis of the cartilage proteome from three different mouse models of genetic skeletal diseases reveals common and discrete disease signatures.Structural and functional characterization of recombinant matrilin-3 A-domain and implications for human genetic bone diseases.Dysplastic histogenesis of cartilage growth plate by alteration of sulphation pathway: a transgenic model.
P2860
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P2860
Decreased chondrocyte proliferation and dysregulated apoptosis in the cartilage growth plate are key features of a murine model of epiphyseal dysplasia caused by a matn3 mutation.
description
2007 nî lūn-bûn
@nan
2007 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի մայիսին 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
Decreased chondrocyte prolifer ...... sia caused by a matn3 mutation
@nl
Decreased chondrocyte prolifer ...... ia caused by a matn3 mutation.
@ast
Decreased chondrocyte prolifer ...... ia caused by a matn3 mutation.
@en
type
label
Decreased chondrocyte prolifer ...... sia caused by a matn3 mutation
@nl
Decreased chondrocyte prolifer ...... ia caused by a matn3 mutation.
@ast
Decreased chondrocyte prolifer ...... ia caused by a matn3 mutation.
@en
prefLabel
Decreased chondrocyte prolifer ...... sia caused by a matn3 mutation
@nl
Decreased chondrocyte prolifer ...... ia caused by a matn3 mutation.
@ast
Decreased chondrocyte prolifer ...... ia caused by a matn3 mutation.
@en
P2093
P2860
P356
P1476
Decreased chondrocyte prolifer ...... ia caused by a matn3 mutation.
@en
P2093
David J Thornton
Farhana Suleman
Karl E Kadler
Lynette Knowles
Matthew P Leighton
Michael D Briggs
Raimund Wagener
Raymond P Boot-Handford
Roger S Meadows
Seema Nundlall
P2860
P304
P356
10.1093/HMG/DDM121
P407
P577
2007-05-21T00:00:00Z