Results 61 to 70 of about 12,218 (212)

Targeted deletion of fibrillin-1 in the mouse eye results in ectopia lentis and other ocular phenotypes associated with Marfan syndrome

open access: yesDisease Models & Mechanisms, 2019
Fibrillin is an evolutionarily ancient protein that lends elasticity and resiliency to a variety of tissues. In humans, mutations in fibrillin-1 cause Marfan and related syndromes, conditions in which the eye is often severely affected.
Wendell Jones   +2 more
doaj   +1 more source

Generation of human induced pluripotent stem cell line UGENTi001-A from a patient with Marfan syndrome carrying a heterozygous c.7754 T > C variant in FBN1 and the isogenic control UGENT001-A-1 using CRISPR/Cas9 editing

open access: yesStem Cell Research, 2023
Marfan syndrome is an autosomal dominant genetic disorder resulting from pathogenic variants in FBN1 gene. FBN1 encodes for fibrillin-1, an important extracellular matrix protein.
Jeffrey Aalders   +7 more
doaj   +1 more source

Innate immunity shapes the persistent transmission of plant viruses by insect vectors

open access: yesNew Plant Protection, EarlyView.
In this review, we focus on two key aspects of the virus–vector interplay: (1) persistently transmitted viruses utilize host factors to overcome transmission barriers; and (2) molecular recognition activates antiviral immunity and subsequent viral counter‐defense. Understanding these interactions offers critical insights for developing novel strategies
Gang Lu, Chuanxi Zhang, Junmin Li
wiley   +1 more source

Progressive Microstructural Deterioration Dictates Evolving Biomechanical Dysfunction in the Marfan Aorta

open access: yesFrontiers in Cardiovascular Medicine, 2021
Medial deterioration leading to thoracic aortic aneurysms arises from multiple causes, chief among them mutations to the gene that encodes fibrillin-1 and leads to Marfan syndrome.
Cristina Cavinato   +7 more
doaj   +1 more source

Extracellular matrix and proteolysis: mechanisms driving irreversible changes and shaping cell behavior

open access: yesThe FEBS Journal, EarlyView.
Irreversible ECM proteolysis by remodeling enzymes shapes development, homeostasis, and disease. ECM‐degrading proteases display cell specificity and are governed by shared mechanisms, exhibiting functional redundancy in generating matrikines, growth factors, and cytokines.
Inna Solomonov, Orit Kollet, Irit Sagi
wiley   +1 more source

Fibrillines et fibrillinopathies [PDF]

open access: yesmédecine/sciences, 1996
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   +2 more sources

Fibrillin-1 mutations causing Weill-Marchesani syndrome and acromicric and geleophysic dysplasias disrupt heparan sulfate interactions.

open access: yesPLoS ONE, 2012
The extracellular glycoprotein fibrillin-1 forms microfibrils that act as the template for elastic fibers. Most mutations in fibrillin-1 cause Marfan syndrome with severe cardiovascular and ocular symptoms, and tall stature.
Stuart A Cain   +4 more
doaj   +1 more source

Higher blood pressure in elderly hypertensive females, with increased arterial stiffness and blood pressure in females with the Fibrillin-1 2/3 genotype

open access: yesBMC Cardiovascular Disorders, 2020
Background Elderly patients have a relatively high cardiovascular risk due to increased arterial stiffness, elevated blood pressure and decreased amounts of elastin in the arteries.
Ida Åström Malm   +4 more
doaj   +1 more source

A guide to the types, structures, and multifaceted functions of matrix metalloproteinases in cancer

open access: yesThe FEBS Journal, EarlyView.
Matrix metalloproteinases (MMPs) orchestrate cancer progression and metastasis through proteolytic and non‐proteolytic actions. By remodeling the tumor microenvironment, enhancing growth factor availability, and modulating cell behavior, MMPs promote proliferation, migration or invasion, and epithelial‐to‐mesenchymal transition. Alongside extracellular
Zoi Piperigkou   +4 more
wiley   +1 more source

Schematic of recombinant fibrillin-1 and fibrillin-2 domain swap fragments and their heparin binding properties.

open access: yes, 2012
(A) Domain structures of fibrillin-1 and fibrillin-2 are shown, with a key of the different domains, N-glycosylation sites, and the C-terminal furin cleavage site. Fibrillin-1 contains a proline-rich region and fibrillin-2 contains a glycine-rich region.
Amanda McGovern (123689)   +4 more
core   +1 more source

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