Results 111 to 120 of about 12,218 (212)

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

Immobilized metal affinity chromatography co-purifies TGF-β1 with histidine-tagged recombinant extracellular proteins.

PLoS ONE, 2012
Extracellular recombinant proteins are commonly produced using HEK293 cells as histidine-tagged proteins facilitating purification by immobilized metal affinity chromatography (IMAC).
Jasvir Kaur, Dieter P Reinhardt
doaj   +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

Fibrillin-1, a calcium binding protein of extracellular matrix.

, 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 ...
P.A Handford, Handford, Penelope, Handford, P.A, Handford, PA   +3 more
core   +1 more source

Fibrillin degradation by matrix metalloproteinases: Identification of amino- and carboxy-terminal cleavage sites

, 1999
Fibrillin molecules form the structural framework of elastic fibrillin-rich microfibrils of the extracellular matrix. We have investigated the proteolysis of recombinant fibrillin molecules by five matrix metalloproteinases.
V.John Hindson, Matthew J Rock, Kielty, Cay M., Jane L Ashworth, Sharon Cunliffe, Rock, Matthew J, Cunliffe, Sharon, Hindson, V. John, Hindson, V.John, Ashworth, Jane L., Rock, Matthew J., Shuttleworth, C.Adrian, Kielty, Cay M, Ashworth, Jane L, Cay M Kielty, Shuttleworth, C. Adrian, C.Adrian Shuttleworth   +16 more
core   +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  

Fibrillin: From microfibril assembly to biomechanical function

, 2002
Fibrillins form the structural framework of a unique and essential class of extracellular microfibrils that endow dynamic connective tissues with long-range elasticity.
Kielty, Cay M., Rock, Matthew J., Ashworth, Jane L., Lee, David, Baldock, Clair, Shuttleworth, C. Adrian   +5 more
core   +1 more source

Genetic and gene expression analyses of the polycystic ovary syndrome candidate gene fibrillin-3 and other fibrillin family members in human ovaries

, 2009
Several studies have demonstrated an association between polycystic ovary syndrome (PCOS) and the dinucleotide repeat microsatellite marker D19S884, which is located in intron 55 of the fibrillin-3 (FBN3) gene.
Prodoehl, MJ, Painter, JN, Prodoehl, Mark, Rainey, William, Irving-Rodgers, HF, Carr, BR, Mason, Helen D., Montgomery, Grant W., Hatzirodos, N, Mason, Helen, Hickey, T., Norman, R., Montgomery, GW, Hatzirodos, Nicholas, Rainey, William E., Painter, Jodie N., Prodoehl, M., Zhao, Zhen Z., Irving-Rodgers, H., Montgomery, G., Painter, J., Hatzirodos, N., Gibson, Mark, Painter, Jodie, Mason, HD, Montgomery, Grant, Norman, RJ, Hickey, Theresa, Hickey, TE, Rodgers, Raymond, Rodgers, RJ, Rainey, W., Carr, Bruce, Irving-Rodgers, Helen, Carr, Bruce R., Carr, B., Prodoehl, Mark J., Norman, Robert, Mason, H., Zhao, ZZ, Gibson, Mark A., Rodgers, R., Rodgers, Raymond J., Gibson, MA, Norman, Robert J., Zhao, Z., Hickey, Theresa E., Zhao, Zhen Zhen, Gibson, M., Rainey, WE, Irving-Rodgers, Helen F.   +50 more
core   +1 more source

Xenopus fibrillin regulates directed convergence and extension

, 2007
Fibrillin-based human diseases such as Marfan syndrome and congenital contractural arachnodactyly implicate fibrillins in the function and homeostasis of multiple adult tissues.
Skoglund, Paul, Keller, Ray
core   +1 more source

Cell Adhesion to Fibrillin-1 Molecules and Microfibrils Is Mediated by α5β1 and αvβ3 Integrins

, 2003
Fibrillins are the major glycoprotein components of microfibrils that form a template for tropoelastin during elastic fibrillogenesis. We have examined cell adhesion to assembled purified microfibrils, and its molecular basis.
Kielty, Cay M., Lomas, Amanda, Humphries, Jon, Morgan, Amanda, Bax, Daniel V., Bernard, Sarah E., Shuttleworth, C. Adrian, Humphries, Martin J.   +7 more
core   +1 more source