Results 71 to 80 of about 10,140 (168)

Behind the scenes: how the EMILIN/Multimerin family shapes the cancer landscape

The FEBS Journal, Volume 293, Issue 13, Page 3991-4013, July 2026.
The EMILIN/Multimerin family members regulate key hallmarks of cancer—including apoptosis, angiogenesis, metastasis, and tumor microenvironment remodeling. As indicated, their function in immune evasion, drug resistance, and metabolic reprogramming remains largely unexplored.
Evelina Poletto, Naike Casagrande, Emanuele Di Siena, Greta Carobolante, Lucrezia Camicia, Giorgia Schinello, Samanta Muzzin, Enrica Timis, Paola Spessotto, Maurizio Mongiat   +9 more
wiley   +1 more source

Proteolysis at the extracellular matrix interface: Molecular architects and regulators in health and disease

The FEBS Journal, Volume 293, Issue 13, Page 3758-3787, July 2026.
The extracellular matrix (ECM) is a dynamic scaffold that orchestrates tissue architecture and cellular communication. A critical but underexplored interplay between proteases and cluster of differentiation molecules (CD) governs ECM turnover and directs cell fate.
David Jurnečka, Matija Hrovatin, Bor Bokalj, Boris Turk   +3 more
wiley   +1 more source

Compound heterozygous mutations in FBN1 in a large family with Marfan syndrome

Molecular Genetics & Genomic Medicine, 2020
Background Marfan syndrome (MFS) is a dominant monogenic disorder caused by mutations in fibrillin 1 (FBN1). Rarely, compound heterozygosity for FBN1 mutations has been described. Methods A large kindred with MFS was assessed clinically over decades, and
Aideen M. McInerney‐Leo, Jennifer West, Lawrie Wheeler, Paul J. Leo, Kim M. Summers, Lisa Anderson, Matthew A. Brown, Malcolm West, Emma L. Duncan   +8 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

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

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

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

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

P1148A in fibrillin-1 is not a mutation anymore [PDF]

Nature Genetics, 1997
M, Wang, K R, Mathews, K, Imaizumi, S, Beiraghi, B, Blumberg, M, Scheuner, J M, Graham, M, Godfrey   +7 more
openaire   +2 more sources