Results 21 to 30 of about 18,239 (220)

Homotypic Fibrillin-1 Interactions in Microfibril Assembly [PDF]

Journal of Biological Chemistry, 2005
We have defined the homotypic interactions of fibrillin-1 to obtain new insights into microfibril assembly. Dose-dependent saturable high affinity binding was demonstrated between N-terminal fragments, between furin processed C-terminal fragments, and between these N- and C-terminal fragments.
Marson, Andrew, Rock, Matthew J., Cain, Stuart A., Freeman, Lyle J., Morgan, Amanda, Mellody, Kieran, Shuttleworth, C. Adrian, Baldock, Clair, Kielty, Cay M.   +8 more
openaire   +3 more sources

Functional Analysis of an Intronic FBN1 Pathogenic Gene Variant in a Family With Marfan Syndrome

Frontiers in Genetics, 2022
Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder that canonically affects the ocular, skeletal, and cardiovascular system, in which aortic tear and rupture is the leading cause of death for MFS patients.
Kui Hu, Yun Wan, Fu-Tsuen Lee, Fu-Tsuen Lee, Jinmiao Chen, Hao Wang, Haonan Qu, Tao Chen, Wang Lu, Zhenwei Jiang, Lufang Gao, Xiaojuan Ji, Liqun Sun, Daokang Xiang   +13 more
doaj   +1 more source

Ca2+-dependent Interface Formation in Fibrillin-1 [PDF]

Journal of Biological Chemistry, 2005
The calcium-binding epidermal growth factor-like (cbEGF) domain is a common structural motif in extracellular and transmembrane proteins. K(d) values for Ca2+ vary from the millimolar to nanomolar range; however the molecular basis for this variation is poorly understood.
Jensen, SA, Corbett, A, Knott, V, Redfield, C, Handford, P   +4 more
openaire   +2 more sources

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

, 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   +2 more sources

Fibrillin-1 regulates the bioavailability of TGFβ1 [PDF]

The 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.
Chaudhry, Shazia S., Cain, Stuart A., Morgan, Amanda, Dallas, Sarah L., Shuttleworth, C. Adrian, Kielty, Cay M.   +5 more
openaire   +2 more sources

Atenolol versus losartan in children and young adults with Marfan's syndrome [PDF]

, 2014
BACKGROUND : Aortic-root dissection is the leading cause of death in Marfan's syndrome. Studies suggest that with regard to slowing aortic-root enlargement, losartan may be more effective than beta-blockers, the current standard therapy in most centers.
Atz, A.M., Backer, J. de, Benson, D.W., Bradley, T.J., Braverman, A.C., Chen, S., Colan, S.D., Dietz, H.C., et al., Gelb, B.D., Grossfeld, P.D., Klein, G.L., Lacro, R.V., Lai, W.W., Levine, J.C., Liou, A., Loeys, B.L., Mahony, L., Markham, L.W., Olson, A.K., Paridon, S.M., Pearson, G.D., Pemberton, V.L., Pierpont, M.E., Pyeritz, R.E., Radojewski, E., Roman, M.J., Sharkey, A.M., Sleeper, L.A., Stylianou, M.P., Tierney, E.S., Wechsler, S.B., Yetman, A.T., Young, L.T.   +33 more
core   +7 more sources

Involvement of Aquaporin 1 in the Motility and in the Production of Fibrillin 1 and Type I Collagen of Cultured Human Dermal Fibroblasts

Cosmetics, 2022
Aminocarbonyl proteins increase with age in the dermal layer. Gene Chip analysis of mRNA expression in human dermal fibroblasts cultured on collagen gels treated with glyceraldehyde as an aminocarbonyl protein and on untreated collagen gels showed a ...
Kazuhisa Maeda, Shiori Yoshida
doaj   +1 more source

1H, 13C and 15N resonance assignments for the fibrillin-1 EGF2-EGF3-hybrid1-cbEGF1 four-domain fragment [PDF]

, 2013
Fibrillins are large extracellular glycoproteins that form the principal component of microfibrils. These perform a vital structural function in the extracellular matrix of many tissues.
Christina Redfield, David A. Yadin, Ian B. Robertson, Isabelle Osuch, Penny A. Handford, Sacha A. Jensen   +5 more
core   +1 more source

Angiotensin II blockade and aortic-root dilation in Marfan's syndrome [PDF]

, 2008
Background: Progressive enlargement of the aortic root, leading to dissection, is the main cause of premature death in patients with Marfan's syndrome. Recent data from mouse models of Marfan's syndrome suggest that aortic-root enlargement is caused by ...
Brooke, Benjamin S., Dietz, Harry C., Habashi, Jennifer P., Judge, Daniel P., Loeys, Bart, Patel, Nishant   +5 more
core   +2 more sources

Calcium Stabilizes Fibrillin-1 against Proteolytic Degradation [PDF]

Journal of Biological Chemistry, 1997
The calcium-binding epidermal growth factor (cbEGF)-like domain is a structural motif that is present in many matrix proteins throughout the animal kingdom from invertebrates to mammals. This module has been demonstrated to bind calcium in the micromolar range.
Dieter P. Reinhardt, Ono, Robert N., Sakai, Lynn Y.   +2 more
openaire   +2 more sources