Results 101 to 110 of about 23,678,950 (225)

Fibrillin-1 regulates periostin expression during maintenance of periodontal homeostasis

Journal of Dental Sciences, 2022
Background/purpose: Human periodontal ligament consists of elastic system fibers, mainly fibrillin-1 (FBN1). Periostin (POSTN) maintains periodontal homeostasis.
Yoshikazu Manabe, Momotoshi Shiga, Kaori Kometani-Gunjigake, Kayoko Nakao-Kuroishi, Masahiro Mizuhara, Takashi Toyono, Yuji Seta, Tatsuo Kawamoto   +7 more
doaj  

Extracellular Matrix‐Mediated Crosslinking of Adhesive Hyaluronic Acid Patch for Treating Volumetric Muscle Injury

Advanced Healthcare Materials, Volume 14, Issue 14, May 28, 2025.
This study reports a novel strategy for preparing a biocompatible adhesive hydrogel crosslinked with a muscle tissue‐derived extracellular matrix (MEM) for in situ muscle regeneration. As a natural crosslinker, MEM not only mediates combinatorial polymer networks but also stimulates satellite cells to induce a cell‐free therapeutic solution.
Eun Je Jeon, Soohwan An, Seung Yeop Han, Eunseon Jeong, Young Seok Song, Jung Seung Lee, Seung‐Woo Cho   +6 more
wiley   +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   +4 more sources

Fibrillin-1, a calcium binding protein of extracellular matrix

Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2000
Fibrillin-1 is a large extracellular matrix glycoprotein which assembles to form 10-12 nm microfibrils in extracellular matrix. Mutations in the human fibrillin-1 gene (FBN-1) cause the connective tissue disease Marfan syndrome and related disorders, which are characterised by defects in the skeletal, cardiovascular and ocular systems of the body ...
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

Multi-exon deletions of the FBN1 gene in Marfan syndrome

BMC Medical Genetics, 2001
Background Mutations in the fibrillin -1 gene (FBN1) cause Marfan syndrome (MFS), an autosomal dominant multi-system connective tissue disorder. The 200 different mutations reported in the 235 kb, 65 exon-containing gene include only one family with a ...
Schrijver Iris, Liu Wanguo, Brenn Thomas, Furthmayr Heinz, Francke Uta   +4 more
doaj   +1 more source

Proteomic fingerprints of damage in extracellular matrix assemblies

Matrix Biology Plus, 2020
In contrast to the dynamic intracellular environment, structural extracellular matrix (ECM) proteins with half-lives measured in decades, are susceptible to accumulating damage.
Alexander Eckersley, Matiss Ozols, Ronan O'Cualain, Emma-Jayne Keevill, April Foster, Suzanne Pilkington, David Knight, Christopher E.M. Griffiths, Rachel E.B. Watson, Michael J. Sherratt   +9 more
doaj  

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

Generation of heterozygous fibrillin-1 mutant cloned pigs from genome-edited foetal fibroblasts

Scientific Reports, 2016
Marfan syndrome (MFS) is an autosomal dominant genetic disease caused by abnormal formation of the extracellular matrix with an incidence of 1 in 3, 000 to 5, 000. Patients with Marfan syndrome experience poor quality of life caused by skeletal disorders
K. Umeyama, Kota Watanabe, Masahito Watanabe, K. Horiuchi, K. Nakano, Masateru Kitashiro, H. Matsunari, Tokuhiro Kimura, Y. Arima, Oltea Sampetrean, Masaki Nagaya, M. Saito, H. Saya, K. Kosaki, H. Nagashima, M. Matsumoto   +15 more
semanticscholar   +1 more source