Fibrillins 1 and 2 Perform Partially Overlapping Functions during Aortic Development [PDF]
Fibrillin-rich microfibrils are extracellular assemblies that impart structural properties to the connective tissue. To elucidate the contribution of fibrillin-rich microfibrils to organogenesis, we have examined the vascular phenotype of a newly created
Luca Carta+12 more
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Latent Transforming Growth Factor β-binding Protein 1 Interacts with Fibrillin and Is a Microfibril-associated Protein [PDF]
Latent transforming growth factor β-binding protein 1 (LTBP-1) targets latent complexes of transforming growth factor β to the extracellular matrix, where the latent cytokine is subsequently activated by several different mechanisms.
Zenzo Isogai+9 more
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The glycoprotein fibrillin is the principal component of the ciliary zonule and has an important role in the strength and elasticity of ocular connective tissues. Fibrillin polymers form the structural scaffold of extensible microfibrils1-3 which are present in ocular elastic tissues and are arranged in parallel bundles to form the zonular fibres.4 ...
Cay M. Kielty+2 more
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Fibrillines et fibrillinopathies [PDF]
Microfibrils contain a variety of proteins, the most prominent of which are the two fibrillins. Fibrillins are large glycoproteins (320 kDa) ubiquitously distributed in connective tissues. Together with amorphous elastin, fibrillin-containing microfibrils form the elastic fibers.
Collod-Beroud, Gwenaëlle+1 more
openaire +3 more sources
Fibrillin: from microfibril assembly to biomechanical function [PDF]
Fibrillins form the structural framework of a unique and essential class of extracellular microfibrils that endow dynamic connective tissues with long–range elasticity. Their biological importance is emphasized by the linkage of fibrillin mutations to Marfan syndrome and related connective tissue disorders, which are associated with severe ...
Cay M. Kielty+5 more
openalex +9 more sources
Transcriptome analysis of skin fibroblasts with dominant negative COL3A1 mutations provides molecular insights into the etiopathology of vascular Ehlers-Danlos syndrome. [PDF]
Vascular Ehlers-Danlos syndrome (vEDS) is a dominantly inherited connective tissue disorder caused by mutations in the COL3A1 gene that encodes type III collagen (COLLIII), which is the major expressed collagen in blood vessels and hollow organs.
Nicola Chiarelli+4 more
doaj +1 more source
Characterization of Microfibrillar-associated Protein 4 (MFAP4) as a Tropoelastin- and Fibrillin-binding Protein Involved in Elastic Fiber Formation [PDF]
MFAP4 (microfibrillar-associated protein 4) is an extracellular glycoprotein found in elastic fibers without a clearly defined role in elastic fiber assembly.
Heumüller, Stefanie E+11 more
core +1 more source
The N-terminal Part of Arabidopsis thaliana Starch Synthase 4 Determines the Localization and Activity of the Enzyme [PDF]
Starch synthase 4 (SS4) plays a specific role in starch synthesis because it controls the number of starch granules synthesized in the chloroplast and is involved in the initiation of the starch granule.
Mérida Berlanga, Ángel+2 more
core +1 more source
Arabidopsis thaliana plastoglobule‐associated fibrillin 1a interacts with fibrillin 1b in vivo [PDF]
Plant fibrillins are a well‐conserved protein family found in the plastids of all photosynthetic organisms, where they perform a wide range of functions. A number of these proteins have been suggested to be involved in the maintenance of thylakoids and the formation of plastoglobules, preventing their coalescence and favoring their clustering via an as‐
Gámez Arjona, Francisco M.+3 more
openaire +5 more sources
Elastin is Localised to the Interfascicular Matrix of Energy Storing Tendons and Becomes Increasingly Disorganised With Ageing [PDF]
Tendon is composed of fascicles bound together by the interfascicular matrix (IFM). Energy storing tendons are more elastic and extensible than positional tendons; behaviour provided by specialisation of the IFM to enable repeated interfascicular sliding
A Heinz+48 more
core +3 more sources