Results 131 to 140 of about 10,140 (168)

Fibrillin-1 regulates the bioavailability of TGFβ1 [PDF]

Journal of Cell Biology, 2007
We have discovered that fibrillin-1, which forms extracellular microfibrils, can regulate the bioavailability of transforming growth factor (TGF) β1, a powerful cytokine that modulates cell survival and phenotype. Altered TGFβ signaling is a major contributor to the pathology of Marfan syndrome (MFS) and related diseases.
Stuart Cain, Sarah Dallas, Cay Kielty
exaly   +3 more sources

In Vivo Studies of Mutant Fibrillin-1 Microfibrils [PDF]

Journal of Biological Chemistry, 2010
In humans, mutations in fibrillin-1 result in a variety of genetic disorders with distinct clinical phenotypes. While most of the known mutations in fibrillin-1 cause Marfan syndrome, a number of other mutations lead to clinical features unrelated to Marfan syndrome. Pathogenesis of Marfan syndrome is currently thought to be driven by mechanisms due to
Francesco Ramirez, Sara Tufa, Lynn Sakai   +2 more
exaly   +3 more sources

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Fibrillin-1: Organization in Microfibrils and Structural Properties

Journal of Molecular Biology, 1996
To investigate the microfibrillar organization and structural properties of fibrillin-1, we produced overlapping recombinant peptides in human cells which altogether span the fibrillin-1 molecule. The peptides were purified under non-denaturing conditions and extensive characterization indicated correct folding.
Dieter Peter Reinhardt, Lynn Sakai, Douglas Keene   +2 more
exaly   +4 more sources

Defective Calcium Binding to Fibrillin-1: Consequence of an N2144S Change for Fibrillin-1 Structure and Function

Journal of Molecular Biology, 1999
Fibrillin-1 is a major structural component of 10-12 nm connective tissue microfibrils and has a modular organisation that includes 43 calcium binding epidermal growth factor-like (cbEGF) domains and seven transforming growth factor beta-binding protein-like (TB) domains.
S, Kettle, X, Yuan, G, Grundy, V, Knott, A K, Downing, P A, Handford   +5 more
openaire   +2 more sources

Fibrillin‐1 in the Vasculature: In Vivo Accumulation of eGFP‐Tagged Fibrillin‐1 in a Knockin Mouse Model

The Anatomical Record, 2019
ABSTRACTImmunolocalization studies have shown that fibrillin‐1 is distributed ubiquitously in the connective tissue space from early embryonic times through old age. When mutated, the gene for fibrillin‐1 (FBN1) causes the Marfan syndrome, a common inherited disorder of connective tissue.
Noe L. Charbonneau, Elise C. Manalo, Sara F. Tufa, Eric J. Carlson, Valerie M. Carlberg, Douglas R. Keene, Lynn Y. Sakai   +6 more
openaire   +2 more sources

Non-Enzymatic Glycation of Human Fibrillin-1

Gerontology, 2008
Non-enzymatic glycation of proteins is one of the key mechanisms in the pathogenesis of diabetic complications and may be significant in the age-related changes of tissues. We isolated and investigated the in vitro glycation of human aortic fibrillin-1. Fibrillin-1 was prepared from thoracic aortas of 9 accident victims distributed in three age groups.
Milena, Atanasova, Emiliana, Konova, Tatiana, Betova, Stephan, Baydanoff   +3 more
openaire   +2 more sources

Fibrillin-1 and asprosin, novel players in metabolic syndrome

Molecular Genetics and Metabolism, 2023
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,
Summers, Kim M., Bush, Stephen J., Davis, Margaret R., Hume, David A., Keshvari, Sahar, West, Jennifer A.   +5 more
openaire   +4 more sources

Regulation of fibrillin-1 gene expression by Sp1

Gene, 2013
Mutations in the fibrillin-1 gene (FBN1) cause Marfan Syndrome (MFS), a hereditary disorder of connective tissue. The transcription of FBN1 has been reported to be driven by a short ultraconserved region (SUPR) in the 5' untranslated exon A of FBN1, but the nature of other factors involved in FBN1 gene regulation has not been clarified.
Gao, Guo, Christian, Rödelsperger, Martin, Digweed, Peter N, Robinson   +3 more
openaire   +2 more sources

Fibrillin-1 im Bindegewebe

2017
Bindegewebe bezeichnet verschiedene Gewebetypen im Korper, die auf unterstutzende Aufgaben spezialisiert sind. Diese Gewebetypen enthalten relativ wenige Korperzellen sowie das biologische Material zwischen den Zellen, die so genannte extrazellulare Matrix.
Karina A. Zeyer, Dieter P. Reinhardt
openaire   +1 more source

PARTIAL CLONING AND SEQUENCING OF CHICK FIBRILLIN-1 cDNA

In Vitro Cellular & Developmental Biology - Animal, 2000
The recent identification of numerous matrix genes and gene products has allowed a detailed examination of their roles in development. Two of these extracellular matrix proteins, fibrillin-1 and fibrillin-2, are components of the elastin-associated microfibrils.
G, Zhou, C E, Price, T H, Rosenquist, P F, Gadson, M, Godfrey   +4 more
openaire   +2 more sources