Results 1 to 10 of about 22,293 (293)

Fibrillin-1 Regulates Arteriole Integrity in the Retina

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, Ling Li, Isabelle Fremaux, Dieter Peter Reinhardt, Elisabeth Génot   +4 more
doaj   +2 more sources

The ADAMTS/Fibrillin Connection: Insights into the Biological Functions of ADAMTS10 and ADAMTS17 and Their Respective Sister Proteases

Biomolecules, 2020
Secreted a disintegrin-like and metalloprotease with thrombospondin type 1 motif (ADAMTS) proteases play crucial roles in tissue development and homeostasis.
Stylianos Z. Karoulias, Nandaraj Taye, Sarah Stanley, Dirk Hubmacher   +3 more
doaj   +2 more sources

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

Disease 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, Juan Rodriguez, Steven Bassnett   +2 more
doaj   +2 more sources

Phosphate overload via the type III Na-dependent Pi transporter represses aortic wall elastic fiber formation [PDF]

Fujita Medical Journal
Objectives: Phosphate (Pi) induces differentiation of arterial smooth muscle cells to the osteoblastic phenotype by inducing the type III Na-dependent Pi transporter Pit-1/solute carrier family member 1.
Yasumasa Yoshino, Tomoka Hasegawa, Shukei Sugita, Eisuke Tomatsu, Naoya Murao, Izumi Hiratsuka, Sahoko Sekiguchi-Ueda, Megumi Shibata, Takeo Matsumoto, Norio Amizuka, Yusuke Seino, Takeshi Takayanagi, Yoshihisa Sugimura, Atsushi Suzuki   +13 more
doaj   +2 more sources

The Biomechanics of Fibrillin Microfibrils: Lessons from the Ciliary Zonule [PDF]

Cells
Marfan syndrome is an inherited connective tissue disorder that affects the cardiovascular, musculoskeletal, and ocular systems. It is caused by pathogenic variants in the fibrillin-1 gene (FBN1).
Pooja Rathaur, Juan Rodriguez, John Kuchtey, Samuel Insignares, Wendell B. Jones, Rachel W. Kuchtey, Steven Bassnett   +6 more
doaj   +2 more sources

ADAR1 Is Essential for Smooth Muscle Homeostasis and Vascular Integrity [PDF]

Cells
Vascular smooth muscle cells (VSMCs) play a critical role in maintaining vascular integrity. VSMC dysfunction leads to numerous vascular diseases. Adenosine deaminases acting on RNA 1 (ADAR1), an RNA editing enzyme, has shown both RNA editing and non ...
Dunpeng Cai, Shi-You Chen
doaj   +2 more sources

The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice

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, Tanja Skrba, Elisabeth Wondimu, Giuseppina Galatioto, René Brüggebusch Svensson, Annesofie T. Olesen, Abigail L. Mackey, S. Peter Magnusson, Francesco Ramirez, Michael Kjaer   +9 more
doaj   +2 more sources

A heart for fibrillin : spatial arrangement in adult wild-type murine myocardial tissue [PDF]

Histochemistry and Cell Biology, 2018
Fibrillins are major constituents of microfibrils, which are essential components of the extracellular matrix of connective tissues where they contribute to the tissue homeostasis.
De Backer, Julie, Renard, Marjolijn, Sips, Patrick, Steijns, Felke, van Hengel, Jolanda   +4 more
core   +3 more sources

Characterization of Microfibrillar-associated Protein 4 (MFAP4) as a Tropoelastin- and Fibrillin-binding Protein Involved in Elastic Fiber Formation [PDF]

Journal of Biological Chemistry, 2015
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, Holm, Anne T, Holmskov, Uffe, Moeller, Jesper B, Moestrup, Soren K, Pilecki, Bartosz, Schlosser, Anders, Sengle, Gerhard, Sorensen, Grith L, Wallis, Russell, Wohl, Alexander P, Zuk, Alexandra V   +11 more
core   +2 more sources

New insights into the structural role of EMILINs within the human skin microenvironment [PDF]

Scientific Reports
Supramolecular extracellular matrix (ECM) networks play an essential role in skin architecture and function. Elastin microfibril interface-located proteins (EMILINs) comprise a family of three extracellular glycoproteins that serve as essential ...
Alvise Schiavinato, Fady Marcous, Alexandra V. Zuk, Douglas R. Keene, Sara F. Tufa, Laura M. Mosquera, Paola Zigrino, Cornelia Mauch, Beate Eckes, Katrien Francois, Julie De Backer, Nicolas Hunzelmann, Pia Moinzadeh, Thomas Krieg, Bert Callewaert, Gerhard Sengle   +15 more
doaj   +2 more sources