An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia.
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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 contributionDefining the extracellular matrix using proteomicsMechanisms and models of endoplasmic reticulum stress in chondrodysplasiaAdvances in Skeletal Dysplasia GeneticsExploring the Conserved Role of MANF in the Unfolded Protein Response in Drosophila melanogasterToll-like receptor-mediated IRE1α activation as a therapeutic target for inflammatory arthritisBiosynthesis and secretion of mouse cysteine-rich with EGF-like domains 2A structure of a collagen VI VWA domain displays N and C termini at opposite sides of the protein.In vivo screening reveals interactions between Drosophila Manf and genes involved in the mitochondria and the ubiquinone synthesis pathway.Role of an ER stress response element in regulating the bidirectional promoter of the mouse CRELD2 - ALG12 gene pair.Transcriptional profiling of chondrodysplasia growth plate cartilage reveals adaptive ER-stress networks that allow survival but disrupt hypertrophyClinical and molecular analysis of UAE fibrochondrogenesis patients expands the phenotype and reveals two COL11A1 homozygous null mutations.Emerging genetic basis of osteochondritis dissecans.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).Changes 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 mutationNew therapeutic targets in rare genetic skeletal 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.Extracellular matrix and developing growth plate.C/EBP homologous protein drives pro-catabolic responses in chondrocytes.Armet/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.Activating the unfolded protein response in osteocytes causes hyperostosis consistent with craniodiaphyseal dysplasia.ER Stress During the Pubertal Growth Spurt Results in Impaired Long-Bone Growth in Chondrocyte-Specific ERp57 Knockout Mice.MED resulting from recessively inherited mutations in the gene encoding calcium-activated nucleotidase CANT1.Inhibiting the integrated stress response pathway prevents aberrant chondrocyte differentiation thereby alleviating chondrodysplasiaExpression analysis and functional characterization of the mouse cysteine-rich with EGF-like domains 2
P2860
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P2860
An unfolded protein response is the initial cellular response to the expression of mutant matrilin-3 in a mouse model of multiple epiphyseal dysplasia.
description
2010 nî lūn-bûn
@nan
2010 թուականի Ապրիլին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի ապրիլին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
An unfolded protein response i ...... multiple epiphyseal dysplasia.
@ast
An unfolded protein response i ...... multiple epiphyseal dysplasia.
@en
type
label
An unfolded protein response i ...... multiple epiphyseal dysplasia.
@ast
An unfolded protein response i ...... multiple epiphyseal dysplasia.
@en
prefLabel
An unfolded protein response i ...... multiple epiphyseal dysplasia.
@ast
An unfolded protein response i ...... multiple epiphyseal dysplasia.
@en
P2093
P2860
P1476
An unfolded protein response i ...... multiple epiphyseal dysplasia
@en
P2093
Benjamin Gardner
Christopher Clowes
Leo A H Zeeff
M Helen Rajpar
Michael D Briggs
Peter A Bell
Seema Nundlall
P2860
P2888
P304
P356
10.1007/S12192-010-0193-Y
P577
2010-04-30T00:00:00Z