Results 171 to 180 of about 23,678,950 (225)

The N-Terminal Region of Fibrillin-1 Mediates a Bipartite Interaction with LTBP1

, 2017
Ian B. Robertson, Hans F. Dias, Isabelle Osuch, E.D. Lowe, Sacha A. Jensen, Christina Redfield, Penny A. Handford   +6 more
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Fibrillin-1 G234D mutation in the hybrid1 domain causes tight skin associated with dysregulated elastogenesis and increased collagen cross-linking in mice.

Matrix Biol
Hossain AS, Clarin MTRDC, Kimura K, Biggin G, Taga Y, Uto K, Yamagishi A, Motoyama E, Narenmandula, Mizuno K, Nakamura C, Asano K, Ohtsuki S, Nakamura T, Kanki S, Baldock C, Raja E, Yanagisawa H.   +17 more
europepmc   +1 more source

Fibrillin‐1 Regulates Skeletal Stem Cell Differentiation by Modulating TGFβ Activity Within the Marrow Niche

Journal of Bone and Mineral Research, 2016
Silvia Smaldone, Nicholas P. Clayton, M. del Solar, G. Pascual, Seng H. Cheng, B. Wentworth, M. Schaffler, F. Ramirez   +7 more
semanticscholar   +1 more source

Role of fibrillin-1 in the cardiovascular function during ageing : fibrillin-1 signalling in human endothelial cells and structural and functional exploration in Fbn-1+/mg delta mice.

, 2009
Boubacar Mariko
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Expression of elastin, FBXW2, fibrillin-1, and α-smooth muscle actin in damaged blood vessels of bovine legs [PDF]

Mari Akiyama
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Sex specific knee joint soft tissue mineralization with Fibrillin-1 mutation in male Tight Skin mice

Keenan C, Wang X, Dikmen T, Wen Y, Ramos-Mucci L, Shorter E, Abraham D, Bou-Gharios G, Poulet B.   +8 more
europepmc   +1 more source

Integrin-mediated mTOR signaling drives TGF-β overactivity and myxomatous mitral valve degeneration in hypomorphic fibrillin-1 mice

Fu Gao, Qixin Chen, Makoto Mori, S. Li, Giovanni Ferrari, Markus Krane, Rong Fan, George Tellides, Yang Liu, Arnar Geirsson   +9 more
openalex   +1 more source

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Fibrillin-1 and asprosin, novel players in metabolic syndrome.

Molecular Genetics and Metabolism, 2022
Fibrillin-1 is a major component of the extracellular microfibrils, where it interacts with other extracellular matrix proteins to provide elasticity to connective tissues, and regulates the bioavailability of TGFβ family members. A peptide consisting of the C-terminal 140 amino acids of fibrillin-1 has recently been identified as a glucogenic hormone,
K. Summers, S. Bush, M. Davis, D. Hume, S. Keshvari, J. West   +5 more
semanticscholar   +3 more sources

The critical role of the TB5 domain of Fibrillin-1 in endochondral ossification.

Human Molecular Genetics, 2022
Mutations in the Fibrillin-1 (FBN1) gene are responsible for the autosomal dominant form of Geleophysic Dysplasia (GD), which is characterized by short stature and extremities, thick skin, and cardiovascular disease.
Laure Delhon, Z. Mougin, Jérémie Jonquet, A. Bibimbou, J. Dubail, Cynthia Bou-Chaaya, Nicolas Goudin, W. Le Goff, C. Boileau, V. Cormier-Daire, C. Le Goff   +10 more
semanticscholar   +1 more source

Fibrillin-1 Misfolding and Disease

Antioxidants and Redox Signaling, 2006
Fibrillin-1 is a 350 kDa calcium-binding protein which assembles to form 10-12 nm microfibrils in the extracellular matrix (ECM). The structure of fibrillin-1 is dominated by two types of disulfide-rich motifs, the calcium- binding epidermal growth factor-like (cbEGF) and transforming growth factor beta binding protein-like (TB) domains.
Whiteman, P, Hutchinson, S, Handford, P
exaly   +5 more sources