Results 111 to 120 of about 22,293 (293)

Pathogenic Mechanisms of Bicuspid Aortic Valve Aortopathy

Frontiers in Physiology, 2017
Bicuspid aortic valve (BAV) is the most common congenital valvular defect and is associated with ascending aortic dilation (AAD) in a quarter of patients. AAD has been ascribed both to the hemodynamic consequences of normally functioning and abnormal BAV
Noor M. Yassine, Jasmine T. Shahram, Simon C. Body   +2 more
doaj   +1 more source

The structural role of elastic fibres in the cornea investigated using a mouse model for Marfan syndrome [PDF]

, 2017
Purpose: The presence of fibrillin-rich elastic fibers in the cornea has been overlooked in recent years. The aim of the current study was to elucidate their functional role using a mouse model for Marfan syndrome, defective in fibrillin-1, the major ...
Bell, James Stephen, Farinha, L., Fergusson, James R., Hayes, Sally, Lewis, Philip, Meek, Keith Michael Andrew, Pereira, L. V., White, Nicholas, White, Tomas   +8 more
core   +1 more source

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

One More Piece in the Fibrillin Puzzle [PDF]

Structure, 2009
Jensen et al. report the crystal structure of a human fibrillin-1 hybrid domain in this issue of Structure. This domain is found exclusively in the fibrillin/latent transforming growth factor-β binding protein superfamily and shares structural features with two other domains in these proteins, the TB/8-Cys and the cbEGF domains.
Dieter P. Reinhardt, Dirk Hubmacher
openaire   +2 more sources

A disease-associated mutation in fibrillin-1 differentially regulates integrin-mediated cell adhesion

Journal of Biological Chemistry, 2019
Fibrillins serve as scaffolds for the assembly of elastic fibers that contribute to the maintenance of tissue homeostasis and regulate growth factor signaling in the extracellular space.
Joselyn S. Del Cid, N. I. Reed, Kathleen S. Molnar, Sean Liu, B. Dang, S. Jensen, W. DeGrado, P. Handford, D. Sheppard, A. Sundaram   +9 more
semanticscholar   +1 more source

CD248 deficiency promotes angiotensin II‐induced aortic lesion by attenuating receptor stability in smooth muscle cells

Clinical and Translational Medicine, Volume 15, Issue 5, May 2025.
1. In the established angiotensin II‐induced AAA model, CD248 deficiency exacerbated aortic lesion, accompanied by lower collagen I content and p38 activation. 2. Silencing CD248 in VSMCs led to reduced MAP kinase activation and ECM production. 3.
Tai‐Tzu Hsieh, Ya‐Chu Ku, Chu‐Jen Chen, Cheng‐Hsiang Kuo, Bi‐Ing Chang, Chien‐Hung Yu, Yi‐Heng Li, Pei‐Jane Tsai, Shu‐Wha Lin, Hua‐Lin Wu, Chwan‐Yau Luo, Yau‐Sheng Tsai   +11 more
wiley   +1 more source

A patient with pleuroparenchymal fibroelastosis carrying a novel fibrillin-2 gene variant

Respiratory Medicine Case Reports, 2023
Pleuroparenchymal fibroelastosis is a recently recognized clinical entity characterized by interstitial pneumonia with proliferating elastin in the upper lung regions. Pleuroparenchymal fibroelastosis is categorized as idiopathic or reported depending on
Kouko Hidaka, Tetsuichiro Inai, Tomoki Kosho, Tomomi Yamaguchi, Yoshinori Kawabata, Yuko Inai, Shogo Imamura, Sakiko Sanada   +7 more
doaj  

Comprehensive clinical and molecular analysis of 12 families with type 1 recessive cutis laxa. [PDF]

, 2013
Autosomal recessive cutis laxa type I (ARCL type I) is characterized by generalized cutis laxa with pulmonary emphysema and/or vascular complications. Rarely, mutations can be identified in FBLN4 or FBLN5.
Bresson, J.L., Callewaert, B., Coucke, P.J., Davis, E.C., De Almeida, S., De Paepe, A., Gaspar, H., Gosendi, M.E., Heimdal, K., Kornak, U., Lee, J.G., Mac Neal, M., Madan-Khetarpal, S., Malfait, F., Mansour, S., Piérard, G.E., Prescott, K., Salhi, A., Schulz, B., Sciurba, F.C., Su, C.T., Symoens, S., Unger, S., Urban, Z., Van Damme, T., Van Maldergem, L., Vanakker, O., Vlummens, P.   +27 more
core   +1 more source

Modeling Stromal Cells Inside the Tumor Microenvironment of Ovarian Cancer: In Vitro Generation of Cancer‐Associated Fibroblast‐Like Cells and Their Impact in a 3D Model

MedComm, Volume 6, Issue 5, May 2025.
Conditioned medium from ovarian cancer cell lines influences fibroblast differentiation into distinct CAF subpopulations (iCAF, myCAF). A 3D model mimicking the tumor microenvironment was developed to study CAF roles in tumor spheroid growth and invasion.
Jacopo Romoli, Paola Chiodelli, Patrizia Bonassi Signoroni, Elsa Vertua, Clarissa Ferrari, Elisabetta Giuzzi, Alice Paini, Elisa Scalvini, Andrea Papait, Francesca Romana Stefani, Antonietta Rosa Silini, Ornella Parolini   +11 more
wiley   +1 more source

Alternative Splicing of FBLN2 Generates a Prometastatic Extracellular Matrix in Gastrointestinal Cancers by Determining N‐Glycosylation of Fibulin 2

Genes to Cells, Volume 30, Issue 3, May 2025.
Fibulin 2 (FBLN2) is an extracellular matrix (ECM) glycoprotein that exists as two alternative splice variants—v1 and v2—with only v1 including the amino acid sequence encoded by exon 9. Whereas the exclusion of FBLN2 exon 9 is one of the most recurrent splicing events across multiple types of cancer, its functional relevance in cancer has remained ...
Ryo Funayama, Yujue Wang, Masaki Hosogane, Wei‐Chen Kao, Shingo Toyama, Masahiro Ohira, Masaki Matsumoto, Takashi Aizawa, Minoru Kobayashi, Hideaki Karasawa, Shinobu Ohnuma, Keiichi I. Nakayama, Michiaki Unno, Keiko Nakayama   +13 more
wiley   +1 more source