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ADAMTS10-mediated tissue disruption in Weill–Marchesani syndrome
The data indicate that decreased SMAD1/5/8 and increased p38/MAPK signalling are associated with ADAMTS10‐induced WMS, and this model will allow further studies of the disease mechanism to facilitate the development of therapeutic interventions.
The role of fibrillin and microfibril binding proteins in elastin and elastic fibre assembly
Fibrillin microfibrils mediate cell signalling via integrin and syndecan receptors, and microfibils sequester transforming growth factor (TGF)β family growth factors within the matrix to provide a tissue store which is critical for homeostasis and remodelling.
Epithelial–mesenchymal status influences how cells deposit fibrillin microfibrils
TGF&bgr;, which stimulates epithelial–mesenchymal transition, altered gene expression and overcame the dependency on syndecan-4 for microfibril deposition in epithelial RPE cells, whereas blocking cadherin interactions disrupted microfibil deposition.
Defining the hierarchical organisation of collagen VI microfibrils at nanometre to micrometre length scales☆
Extracellular matrix microfibrils are critical components of connective tissues with a wide range of mechanical and cellular signalling functions. Collagen VI is a heteromeric network-forming
Internal cleavage and synergy with twisted gastrulation enhance BMP inhibition by BMPER
It is shown that human BMPER binds to Tsg through the N-terminal BMP-binding region which alone more potently inhibits B MP-4 signalling than full-length B MPER, suggesting a synergistic function to dampen BMP activity.
Multiscale Imaging Reveals the Hierarchical Organization of Fibrillin Microfibrils
By combining multiple imaging techniques, three levels of hierarchical organization of fibrillin structure are visualize, ranging from micro-scale fiber bundles in the ciliary zonule to nano-scale individual microfibrils, spanning nano- to macro-length scales.
Dual role of the active site ‘lid’ regions of protochlorophyllide oxidoreductase in photocatalysis and plant development
It is shown that Pchlide binding triggers formation of large oligomers of POR using size exclusion chromatography and this structure shows that oligomer formation is most likely driven by the interaction of amino acid residues in the highly conserved lid regions.
A new MMP‐mediated prodomain cleavage mechanism to activate bone morphogenetic proteins from the extracellular matrix
A new MMP‐dependent BMP‐7 activation mechanism from ECM‐targeted pools via specific PD degradation is described, and sandwich ELISA and solid phase cleavage studies in combination with bioactivity assays, single particle TEM, and in silico molecular docking experiments provided evidence that PD cleavage by MMP­13 leads to BMP•7 CPLX disintegration and bioactive GF release.
A multilevel approach to define the hierarchical organisation of extracellular matrix microfibrils
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Structural studies of elastic fibre and microfibrillar proteins
Some of the matrix proteins found in elastic fibres and the new emerging techniques that can shed light on their structure and dynamic properties are described.