New insights into the assembly of extracellular microfibrils from the analysis of the fibrillin 1 mutation in the tight skin mouse.
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Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndromeRegulation of limb patterning by extracellular microfibrilsAbsence of autoantibodies against correctly folded recombinant fibrillin-1 protein in systemic sclerosis patientsGenetic and functional linkage between ADAMTS superfamily proteins and fibrillin-1: a novel mechanism influencing microfibril assembly and functionMicrofibril-associated MAGP-2 stimulates elastic fiber assemblyRadiation-induced skin injury in the animal model of scleroderma: implications for post-radiotherapy fibrosisAn ADAMTSL2 founder mutation causes Musladin-Lueke Syndrome, a heritable disorder of beagle dogs, featuring stiff skin and joint contractures.A new mouse model for marfan syndrome presents phenotypic variability associated with the genetic background and overall levels of Fbn1 expression.Mutations in fibrillin-1 cause congenital scleroderma: stiff skin syndrome.Differential effects of alendronate and losartan therapy on osteopenia and aortic aneurysm in mice with severe Marfan syndromeFibrillin assemblies: extracellular determinants of tissue formation and fibrosisFibrillin microfibrils in bone physiology.Sequestration of latent TGF-β binding protein 1 into CADASIL-related Notch3-ECD depositsFibrillins 1 and 2 perform partially overlapping functions during aortic development.CD19-dependent B lymphocyte signaling thresholds influence skin fibrosis and autoimmunity in the tight-skin mouseChronic obstructive pulmonary disease * 3: Experimental animal models of pulmonary emphysema.B-lymphocyte depletion reduces skin fibrosis and autoimmunity in the tight-skin mouse model for systemic sclerosisFalse cell lines: The problem and a solutionExtracellular microfibrils in vertebrate development and disease processes.Animal models in chronic obstructive pulmonary disease-an overview.Molecular and cellular basis of scleroderma.Fibrillin-1 Regulates Skeletal Stem Cell Differentiation by Modulating TGFβ Activity Within the Marrow Niche.Latent TGF-beta binding protein LTBP-2 decreases fibroblast adhesion to fibronectinThe supramolecular organization of fibrillin-rich microfibrils.Elevated expression of isopeptide bond cross-links contributes to fibrosis in scleroderma and the healing wounds of tight skin mice.Structural and compositional diversity of fibrillin microfibrils in human tissues.Enlarged Optic Nerve Axons and Reduced Visual Function in Mice with Defective Microfibrils
P2860
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P2860
New insights into the assembly of extracellular microfibrils from the analysis of the fibrillin 1 mutation in the tight skin mouse.
description
2000 nî lūn-bûn
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2000年の論文
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2000年学术文章
@wuu
2000年学术文章
@zh-cn
2000年学术文章
@zh-hans
2000年学术文章
@zh-my
2000年学术文章
@zh-sg
2000年學術文章
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2000年學術文章
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2000年學術文章
@zh-hant
name
New insights into the assembly ...... ation in the tight skin mouse.
@ast
New insights into the assembly ...... ation in the tight skin mouse.
@en
type
label
New insights into the assembly ...... ation in the tight skin mouse.
@ast
New insights into the assembly ...... ation in the tight skin mouse.
@en
prefLabel
New insights into the assembly ...... ation in the tight skin mouse.
@ast
New insights into the assembly ...... ation in the tight skin mouse.
@en
P2093
P2860
P356
P1476
New insights into the assembly ...... ation in the tight skin mouse.
@en
P2093
P2860
P304
P356
10.1083/JCB.150.3.667
P407
P577
2000-08-01T00:00:00Z