Results 131 to 140 of about 19,229 (251)

Extracellular Matrix Molecules Facilitating Vascular Biointegration [PDF]

, 2014
All vascular implants, including stents, heart valves and graft materials exhibit suboptimal biocompatibility that significantly reduces their clinical efficacy.
Michael, Praveesuda, Ng, Martin K.C., Waterhouse, Anna, Wise, Steven G.   +3 more
core   +1 more source

Pericellular colocalisation and interactive properties of type VI collagen and perlecan in the intervertebral disc [PDF]

, 2016
The aim of this study was to immunolocalise type VI collagen and perlecan and determine their interactive properties in the intervertebral disc (IVD).
AJ Hayes, CB Little, CC Shu, J Melrose, JM Whitelock, MS Lord, null null   +6 more
core   +3 more sources

Aqueous humor TGFβ and fibrillin-1 in Tsk mice reveal clues to POAG pathogenesis

Scientific Reports
Aqueous humor (AH) and blood levels of transforming growth factor β (TGFβ) are elevated in idiopathic primary open angle glaucoma (POAG) representing a disease biomarker of unclear status and function.
James C. Tan, MinHee K. Ko, Jeong-Im Woo, Kenneth L. Lu, Jonathan A. Kelber   +4 more
doaj   +1 more source

Fibrillin, a new 350-kD glycoprotein, is a component of extracellular microfibrils. [PDF]

, 1986
Lynn Y. Sakai, Douglas R. Keene, Eva Engvall   +2 more
openalex   +1 more source

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

Two mutations in Marfan syndrome resulting in truncated fibrillin polypeptides. [PDF]

, 1992
Katariina Kainulainen, Lynn Y. Sakai, Anne H. Child, F M Pope, Lea Puhakka, Lasse Ryhänen, Aarno Palotie, Ilkka Kaitila, Leena Peltonen   +8 more
openalex   +1 more source

Studies on TGF-β family signalling in the diseased lung tissue [PDF]

, 2012
This thesis focuses on transforming growth factor (TGF)-β signalling system in the human lung. Two lung parenchymal diseases, idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD), were studied.
Leppäranta, Outi
core  

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  

Genomic organization of the sequence coding for fibrillin, the defective gene product in Marfan syndrome [PDF]

, 1993
Lygia da Veiga Pereira, Marina D’Alessio, Francesco Ramirez, Jennifer Lynch, Bryan Sykes, Theresa Pangilinan, J Bonadio   +6 more
openalex   +1 more source

Fluid dynamics of aortic root dilation in Marfan syndrome

, 2014
Aortic root dilation and propensity to dissection are typical manifestations of the Marfan Syndrome (MS), a genetic defect leading to the degeneration of the elastic fibres. Dilation affects the structure of the flow and, in turn, altered flow may play a
Costantini, Martina, Espa, Stefania, Fortini, Stefania, Querzoli, Giorgio, Sorgini, Francesca   +4 more
core   +1 more source