P3.11 INCREASED CAROTID PLAQUE OCCURRENCE IN MEN WITH THE FIBRILLIN-1 2–3 GENOTYPE
Artery Research, 2009 Background: Fibrillin-1 is an important constituent of the vascular wall and earlier studies have indicated an effect of the fibrillin-1 2–3 genotype on blood pressure as well as aortic stiffness.
R. DeBasso +4 more
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Processing and assembly of fibrillin‐1
International Journal of Experimental Pathology, 2000 Fibrillins are extracellular matrix proteins of ˜350 kD. They have a complex multidomain structure and are the major components of microfibrillar structures in both elastic and nonelastic tissues (Sakai et al. 1986; Kielty & Shuttleworth 1995). Mutations in the FBN-1 gene cause Marfan Syndrome, which is a heritable connective tissue disease with an ...
C. Adrian Shuttleworth +2 more
openaire +2 more sources
PLoS ONE, 2012 Extracellular recombinant proteins are commonly produced using HEK293 cells as histidine-tagged proteins facilitating purification by immobilized metal affinity chromatography (IMAC).
Jasvir Kaur, Dieter P Reinhardt
doaj +1 more source
Fibrillin-1-regulated miR-122 has a critical role in thoracic aortic aneurysm formation
Cellular and Molecular Life Sciences, 2022 Rong-Mo Zhang +6 more
semanticscholar +1 more source
Fell‐Muir Lecture: Fibrillin microfibrils: structural tensometers of elastic tissues?
International journal of experimental pathology (Print), 2017 Fibrillin microfibrils are indispensable structural elements of connective tissues in multicellular organisms from early metazoans to humans. They have an extensible periodic beaded organization, and support dynamic tissues such as ciliary zonules that ...
C. Kielty
semanticscholar +1 more source
Delivery of sTRAIL variants by MSCs in combination with cytotoxic drug treatment leads to p53-independent enhanced antitumor effects [PDF]
, 2013 Mesenchymal stem cells (MSCs) are able to infiltrate tumor tissues and thereby effectively deliver gene therapeutic payloads. Here, we engineered murine MSCs (mMSCs) to express a secreted form of the TNF-related apoptosis-inducing ligand (TRAIL), which ...
A Ashkenazi +46 more
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Generation of heterozygous fibrillin-1 mutant cloned pigs from genome-edited foetal fibroblasts
Scientific Reports, 2016 Marfan syndrome (MFS) is an autosomal dominant genetic disease caused by abnormal formation of the extracellular matrix with an incidence of 1 in 3, 000 to 5, 000. Patients with Marfan syndrome experience poor quality of life caused by skeletal disorders
K. Umeyama +15 more
semanticscholar +1 more source
Aqueous humor TGFβ and fibrillin-1 in Tsk mice reveal clues to POAG pathogenesis
Scientific ReportsAqueous humor (AH) and blood levels of transforming growth factor β (TGFβ) are elevated in idiopathic primary open angle glaucoma (POAG) representing a disease biomarker of unclear status and function.
James C. Tan +4 more
doaj +1 more source
Multi-exon deletions of the
BMC Medical Genetics, 2001 Background Mutations in the fibrillin -1 gene (FBN1) cause Marfan syndrome (MFS), an autosomal dominant multi-system connective tissue disorder. The 200 different mutations reported in the 235 kb, 65 exon-containing gene include only one family with a ...
Schrijver Iris +4 more
doaj +1 more source
Nanopatterned acellular valve conduits drive the commitment of blood-derived multipotent cells [PDF]
, 2016 Considerable progress has been made in recent years toward elucidating the correlation among nanoscale topography, mechanical properties, and biological behavior of cardiac valve substitutes.
Aguiari, Paola +11 more
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