Fibrillin-1 Regulates Arteriole Integrity in the Retina [PDF]
Biomolecules, 2022 Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils that provide critical functions in large blood vessels and other tissues.
Florian Alonso +4 more
doaj +5 more sources
The extracellular matrix glycoprotein fibrillin-1 in health and disease [PDF]
Frontiers in Cell and Developmental Biology, 2023 Fibrillin-1 (FBN1) is a large, cysteine-rich, calcium binding extracellular matrix glycoprotein encoded by FBN1 gene. It serves as a structural component of microfibrils and provides force-bearing mechanical support in elastic and nonelastic connective ...
Li Li +5 more
doaj +5 more sources
Fibrillin-1 and fibrillin-1-derived asprosin in adipose tissue function and metabolic disorders [PDF]
Journal of Cell Communication and Signaling, 2020 The extracellular matrix microenvironment of adipose tissue is of critical importance for the differentiation, remodeling and function of adipocytes. Fibrillin-1 is one of the main components of microfibrils and a key player in this process. Furin processing of profibrillin-1 results in mature fibrillin-1 and releases the C-terminal propeptide as a ...
Dieter P Reinhardt
exaly +7 more sources
Fibrillin-1 regulates endothelial sprouting during angiogenesis. [PDF]
Proc Natl Acad Sci U S A, 2023 Significance Mutations in the gene coding for fibrillin-1, a protein in the extracellular matrix, cause Marfan syndrome, which is characterized by pathological changes in large blood vessels. We show that fibrillin-1 regulates angiogenesis, the formation
Alonso F +7 more
europepmc +4 more sources
Case report: Biochemical and clinical phenotypes caused by cysteine substitutions in the epidermal growth factor-like domains of fibrillin-1 [PDF]
Frontiers in Genetics, 2022 Marfan syndrome, an autosomal dominant disorder of connective tissue, is primarily caused by mutations in the fibrillin-1 (FBN1) gene, which encodes the protein fibrillin-1.
Xin Liu +6 more
doaj +3 more sources
Cooperative Mechanism of ADAMTS/ ADAMTSL and Fibrillin-1 in the Marfan Syndrome and Acromelic Dysplasias [PDF]
Frontiers in Genetics, 2021 The term “fibrillinopathies” gathers various diseases with a wide spectrum of clinical features and severity but all share mutations in the fibrillin genes.
Pauline Arnaud +5 more
doaj +3 more sources
The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice [PDF]
Physiological Reports, 2019 Fibrillin‐1 mutations cause pathological changes in connective tissue that constitute the complex phenotype of Marfan syndrome. In this study, we used fibrillin‐1 hypomorphic and haploinsufficient mice (Fbn1mgr/mgR and Fbn1+/− mice, respectively) to ...
Peter H. T. Tran +9 more
doaj +3 more sources
Aortic Strain Correlates with Elastin Fragmentation in Fibrillin-1 Hypomorphic Mice. [PDF]
Circ Rep, 2019 Background: High-frequency ultrasound has facilitated in vivo measurement of murine ascending aorta, allowing aortic strain to be determined from 2-D imaging.
Chen JZ +5 more
europepmc +4 more sources
Assessment of Bones Deficient in Fibrillin-1 Microfibrils Reveals Pronounced Sex Differences. [PDF]
Int J Mol Sci, 2019 Defects in the extracellular matrix protein fibrillin-1 that perturb transforming growth factor beta (TGFβ) bioavailability lead to Marfan syndrome (MFS).
Altinbas L +8 more
europepmc +4 more sources
Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta. [PDF]
Int J Mol Sci, 2023 Fibrillin-1 microfibrils are essential elements of the extracellular matrix serving as a scaffold for the deposition of elastin and endowing connective tissues with tensile strength and elasticity.
Șulea CM +9 more
europepmc +2 more sources