Results 21 to 30 of about 15,376 (205)

Is There a Relationship Between Pelvic Organ Prolapse and Tissue Fibrillin-1 Levels? [PDF]

International Neurourology Journal, 2015
Purpose: Pelvic organ prolapse is a multifactorial disorder in which extracellular matrix defects are implicated. Fibrillin-1 level is reduced in stress urinary incontinence.
Ayla Eser, Eylem Unlubilgin, Fatih Hizli, Muradiye Acar, Zeynep Kamalak, Aydin Kosus, Nermin Kosus, Deniz Hizli, Esra Gunduz   +8 more
doaj   +1 more source

Role of fibrilins in human cancer: A narrative review

Health Science Reports, 2023
Background Fibrillin is one of the extracellular matrix glycoproteins and participates in forming microfibrils found in many connective tissues. The microfibrils enable the elasticity and stretching properties of the ligaments and support connective ...
Mahsa Mahdizadehi, Marie Saghaeian Jazi, Seyyed Mostafa Mir, Seyyed Mehdi Jafari   +3 more
doaj   +1 more source

Neonatal Marfan syndrome: a case report of a novel fibrillin 1 mutation, with genotype-phenotype correlation and brief review of the literature. [PDF]

Ital J Pediatr
Pugnaloni F, De Rose DU, Digilio MC, Magliozzi M, Braguglia A, Valfrè L, Toscano A, Dotta A, Di Pede A.   +8 more
europepmc   +3 more sources

A disease-associated mutation in fibrillin-1 differentially regulates integrin-mediated cell adhesion. [PDF]

J Biol Chem, 2019
Del Cid JS, Reed NI, Molnar K, Liu S, Dang B, Jensen SA, DeGrado W, Handford PA, Sheppard D, Sundaram AB.   +9 more
europepmc   +3 more sources

Microenvironmental regulation by fibrillin-1. [PDF]

PLoS Genetics, 2012
Fibrillin-1 is a ubiquitous extracellular matrix molecule that sequesters latent growth factor complexes. A role for fibrillin-1 in specifying tissue microenvironments has not been elucidated, even though the concept that fibrillin-1 provides ...
Gerhard Sengle, Ko Tsutsui, Douglas R Keene, Sara F Tufa, Eric J Carlson, Noe L Charbonneau, Robert N Ono, Takako Sasaki, Mary K Wirtz, John R Samples, Liselotte I Fessler, John H Fessler, Kiyotoshi Sekiguchi, Susan J Hayflick, Lynn Y Sakai   +14 more
doaj   +1 more source

Fibrillin-1 mutations causing Weill-Marchesani syndrome and acromicric and geleophysic dysplasias disrupt heparan sulfate interactions. [PDF]

PLoS ONE, 2012
The extracellular glycoprotein fibrillin-1 forms microfibrils that act as the template for elastic fibers. Most mutations in fibrillin-1 cause Marfan syndrome with severe cardiovascular and ocular symptoms, and tall stature.
Stuart A Cain, Amanda McGovern, Andrew K Baldwin, Clair Baldock, Cay M Kielty   +4 more
doaj   +1 more source

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

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   +5 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

Generation of human induced pluripotent stem cell line UGENTi001-A from a patient with Marfan syndrome carrying a heterozygous c.7754 T > C variant in FBN1 and the isogenic control UGENT001-A-1 using CRISPR/Cas9 editing

Stem Cell Research, 2023
Marfan syndrome is an autosomal dominant genetic disorder resulting from pathogenic variants in FBN1 gene. FBN1 encodes for fibrillin-1, an important extracellular matrix protein.
Jeffrey Aalders, Laurens Léger, Anthony Demolder, Laura Muiño Mosquera, Paul Coucke, Björn Menten, Julie De Backer, Jolanda van Hengel   +7 more
doaj   +1 more source