Results 131 to 140 of about 10,140 (167)

Fibrillin-1 Deficiency Perturbs Aortic Cholinergic Relaxation and Adrenergic Contraction in a Mouse Model of Early Onset Progressively Severe Marfan Syndrome.

J Vasc Res
Cantalupo A, Asano K, Dikalov S, Gordon D, Ramirez F.   +4 more
europepmc   +1 more source

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

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

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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

Immunohistochemical expression of fibrillin‐1 and fibrillin‐2 during tooth development

Journal of Periodontal Research, 2014
Background and ObjectiveOxytalan fibers are categorized as a microfibril assembly without elastin deposition, and are unique components in the periodontal ligament (PDL). However, little is known about their formation during PDL development. To clarify the mechanisms of oxytalan fiber formation in developing PDL, we performed immunohistochemical ...
M, Kira-Tatsuoka, K, Oka, E, Tsuruga, M, Ozaki, Y, Sawa   +4 more
openaire   +2 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

Gene Expression and Accumulation of Fibrillin-1, Fibrillin-2, and Tropoelastin in Cultured Periodontal Fibroblasts

Journal of Dental Research, 2002
The elastic system fibers consist of three types—oxytalan, elaunin, and elastic fibers—differing in their relative microfibril and elastin contents. All three types are found in human gingiva, but human periodontal ligaments contain only elastin-free fibers.
E, Tsuruga, K, Irie, T, Yajima
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
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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

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