Results 101 to 110 of about 21,236,271 (159)

Isolation of rat fibrillin-1 cDNA and its relevance in metanephric development

, 1998
The role of fibrillin-1 in metanephrogenesis was investigated. Fibrillin-1 cDNA was isolated from the rat kidney cDNA library and sequenced, and its spatiotemporal expression was studied.
Jun Wada, Qiwei Yang, Anil Kumar, Darryl R. Peterson, Kosuke Ota, Naoki Kashihara, Yashpal S. Kanwar   +6 more
core   +1 more source

New insights into the structural role of EMILINs within the human skin microenvironment

Scientific Reports
Supramolecular extracellular matrix (ECM) networks play an essential role in skin architecture and function. Elastin microfibril interface-located proteins (EMILINs) comprise a family of three extracellular glycoproteins that serve as essential ...
Alvise Schiavinato, Fady Marcous, Alexandra V. Zuk, Douglas R. Keene, Sara F. Tufa, Laura M. Mosquera, Paola Zigrino, Cornelia Mauch, Beate Eckes, Katrien Francois, Julie De Backer, Nicolas Hunzelmann, Pia Moinzadeh, Thomas Krieg, Bert Callewaert, Gerhard Sengle   +15 more
doaj   +1 more source

Extracellular Matrix Molecules Facilitating Vascular Biointegration

Journal of Functional Biomaterials, 2012
All vascular implants, including stents, heart valves and graft materials exhibit suboptimal biocompatibility that significantly reduces their clinical efficacy.
Martin K.C. Ng, Praveesuda Michael, Anna Waterhouse, Steven G. Wise   +3 more
doaj   +1 more source

NMR studies of cbEFG-like domains from human fibrillin-1

, 2000
The calcium binding epidermal growth factor-like (cbEGF) 12-13 domain pair from human fibrillin-1 was the focus of studies for this dissertation. Various nuclear magnetic resonance (NMR) spectroscopy techniques were employed to analyse the calcium ...
Kristy Downing, Downing, A. K., Smallridge, Rachel.   +2 more
core   +2 more sources

Downregulation of S100a8 mitigates fibrosis in skeletal muscles affected by Marfan syndrome

Molecular Therapy: Nucleic Acids
This study investigated the potential of eccentric training in attenuating the deleterious effects of Marfan syndrome on skeletal muscles. Marfan syndrome and wild-type mice were divided into a trained and a control group; the former performed a downhill
Audrei R. Santos, Marcelo G. Pereira, Andrei Rozanski, Cesar S. Fuziwara, Tabata L. Nascimento, Lygia V. Pereira, Marcelo S. Aoki, Elen H. Miyabara   +7 more
doaj   +1 more source

Molecular evaluation of the great vessels of patients with bicuspid aortic valve disease Análise molecular dos grandes vasos da base em portadores de valva aórtica bicúspide

Brazilian Journal of Cardiovascular Surgery, 2003
PURPOSE: Bicuspid aortic valve (BAV) is associated with increased prevalence of annulo-aortic ectasia, dissection and ascending aortic aneurysm. This study was undertaken to compare the amount of fibrillin-1 and elastin in the media of great vessels of ...
Mauro Paes Leme, Tirone E. David, Jagdish Butany, Diponkar Banerjee, Eduardo S. Bastos, Sylvio C. Provenzano, Leôncio A. Feitosa, Henrique Murad, Monica M. Ferreira Magnanini   +8 more
doaj   +1 more source

Morphologische Charakterisierung des renalen Phänotyps der Fibrillin-1-Knockout Maus

, 2011
The Marfansyndrome is an autosomal dominant inherited disease. The cause of the Marfansyndrome is a Fibrillin-1 mutation. Fibrillin-1 is the main constituent of the microfibrils. 20-30 % of Marfansyndrome are based on new mutations of this gene.
Ketecioglu, Cigdem
core  

Hammerhead ribozyme-mediated silencing of the mutant fibrillin-1 of tight skin mouse: Insight into the functional role of mutant fibrillin-1

Experimental Cell Research, 2006
The tight skin (Tsk/+) mouse is a model for fibrotic disorders. The genetic defect in the Tsk/+ is an in-frame duplication between exons 17 and 40 of the fibrillin-1 gene which gives rise to a large transcript and protein. Mice homozygous for the mutation die in utero, whereas heterozygotes survive and spontaneously develop connective tissue disease ...
Menon, R, Menon, MR, Shi-Wen, X, Renzoni, E, Bou-Gharios, G, Black, C, Abraham, D   +6 more
openaire   +3 more sources

Fibrillin microfibrils

, 2005
Fibrillin microfibrils are widely distributed extracellular matrix assemblies that endow elastic and nonelastic connective tissues with long-range elasticity. They direct tropoelastin deposition during elastic fibrillogenesis and form an outer mantle for
Cay M. Kielty, Kielty, Cay M., Marson, Andrew, Clair Baldock, Sherratt, Michael J.; id_orcid, Baldock, Clair, Michael.J. Sherratt, Andrew Marson   +7 more
core   +1 more source

Structural and functional failure of fibrillin‑1 in human diseases (Review)

International Journal of Molecular Medicine, 2017
Fibrillins (FBNs) are key relay molecules that form the backbone of microfibrils in elastic and non‑elastic tissues. Interacting with other components of the extracellular matrix (ECM), these ubiquitous glycoproteins exert pivotal roles in tissue development, homeostasis and repair.
Schrenk, Sandra, Cenzi, Carola, Bertalot, Thomas, Conconi, Maria Teresa, Di Liddo, Rosa   +4 more
openaire   +5 more sources