Results 191 to 200 of about 18,239 (220)

Fibrillin-1 mutations in Marfan syndrome and other type-1 fibrillinopathies

Human Mutation, 1997
Fibrillin is the major component of extracellular microfibrils and is widely distributed in connective tissue throughout the body. Mutations in the fibrillin-1 (FBN1) gene, on chromosome 15q21.1, have been found to cause Marfan syndrome, a dominantly inherited disorder characterised by clinically variable skeletal, ocular, and cardiovascular ...
C, Hayward, D J, Brock
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

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

Fibrillin-1 Protein in Tight Skin Mice and Scleroderma

Clinical Reviews in Allergy & Immunology, 2000
Scleroderma (SSc) is a complex and clinically heterogenous disease of unknown origin, in which both genetic and environmental factors appear to be implicated. In addition to skin, other internal organs, including lung, heart, intestine, and kidney, can be affected.
C, Bona, S, Saito
openaire   +2 more sources

Relaxin modulates fibrillin-2, but not fibrillin-1, gene expression by human dermal fibroblasts

2001
Fibrillins-1 and -2 are the main constituents of extracellular elastic microfibrils and contribute significantly to tissue integrity and elasticity [1]. Fibrillin-1 (FBN-1) was first discovered from the medium of cultured human dermal fibroblasts [2] and was identified as part of elastic-associated and non-elastic tissue microfibrils [3,4].
Chrishan S. Samuel, Lynn Y. Sakai, Edward P. Amento   +2 more
openaire   +1 more source

Fibrillin-1

1998
Shirley Ayad, Ray Boot-Handford, Martin J. Humphries, Karl E. Kadler, Adrian Shuttleworth   +4 more
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Immunohistochemical expression of Fibrillin-1 in idiopathic epiretinal membranes

Graefe's Archive for Clinical and Experimental Ophthalmology
To investigate the expression patterns of Fibrillin-1 in idiopathic epiretinal membranes (iERM) and identify Fibrillin-1-secreting cells.iERM samples were collected via standard 27-gauge vitrectomy and subsequently subjected to flat-mount immunohistochemistry with double staining for the following markers: Fibrillin-1, glial acidic fibrillary protein ...
Luu Viet Tien, Manabu Yamamoto, Mizuki Tagami, Norihiko Misawa, Shigeru Honda   +4 more
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Fibrillin-1

2020
openaire   +1 more source

Basic Components of Connective Tissues and Extracellular Matrix: Elastin, Fibrillin, Fibulins, Fibrinogen, Fibronectin, Laminin, Tenascins and Thrombospondins

Advances in Experimental Medicine and Biology, 2014
Jaroslava Halper, Michael Kjaer
exaly  

The Role of Fibrillin 1 and Fibrillin 2 in Severe Scoliosis

1999
Child A, Aubry M., Brice G., Park E., Putnam E., Milewicz D.   +5 more
openaire   +1 more source