Structural and functional characterization of recombinant matrilin-3 A-domain and implications for human genetic bone diseases.
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 contributionMatrilin-3 Role in Cartilage Development and OsteoarthritisStructural and functional features of a collagen-binding matrix protein from the mussel byssusMultiple epiphyseal dysplasiaMatrilin-3 inhibits chondrocyte hypertrophy as a bone morphogenetic protein-2 antagonist.Mild myopathy is associated with COMP but not MATN3 mutations in mouse models of genetic skeletal diseases.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).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 mutationMatrilin-3 chondrodysplasia mutations cause attenuated chondrogenesis, premature hypertrophy and aberrant response to TGF-β in chondroprogenitor cellsCollagen VI, conformation of A-domain arrays and microfibril architecture.Structural and functional investigations of Matrilin-1 A-domains reveal insights into their role in cartilage ECM assembly.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.The NC2 domain of collagen IX provides chain selection and heterotrimerization.Matrilin-3 codelivery with adipose-derived mesenchymal stem cells promotes articular cartilage regeneration in a rat osteochondral defect model.Trio Clinical Exome Sequencing in a Patient With Multicentric Carpotarsal Osteolysis Syndrome: First Case Report in the Balkans.Matrilin-3 as a putative effector of C-type natriuretic peptide signaling during TGF-β induced chondrogenic differentiation of mesenchymal stem cells.
P2860
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P2860
Structural and functional characterization of recombinant matrilin-3 A-domain and implications for human genetic bone diseases.
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年学术文章
@wuu
2007年学术文章
@zh
2007年学术文章
@zh-cn
2007年学术文章
@zh-hans
2007年学术文章
@zh-my
2007年学术文章
@zh-sg
2007年學術文章
@yue
2007年學術文章
@zh-hant
name
Structural and functional char ...... r human genetic bone diseases.
@en
Structural and functional char ...... r human genetic bone diseases.
@nl
type
label
Structural and functional char ...... r human genetic bone diseases.
@en
Structural and functional char ...... r human genetic bone diseases.
@nl
prefLabel
Structural and functional char ...... r human genetic bone diseases.
@en
Structural and functional char ...... r human genetic bone diseases.
@nl
P2093
P2860
P50
P356
P1476
Structural and functional char ...... or human genetic bone diseases
@en
P2093
Leena Ala-Kokko
Michael D Briggs
Paul Coffey
Roger S Meadows
Thomas A Jowitt
P2860
P304
34634-34643
P356
10.1074/JBC.M705301200
P407
P577
2007-09-18T00:00:00Z