Results 31 to 40 of about 15,376 (205)

1H, 13C and 15N resonance assignments for the fibrillin-1 EGF2-EGF3-hybrid1-cbEGF1 four-domain fragment [PDF]

, 2013
Fibrillins are large extracellular glycoproteins that form the principal component of microfibrils. These perform a vital structural function in the extracellular matrix of many tissues.
Christina Redfield, David A. Yadin, Ian B. Robertson, Isabelle Osuch, Penny A. Handford, Sacha A. Jensen   +5 more
core   +1 more source

Progressive Microstructural Deterioration Dictates Evolving Biomechanical Dysfunction in the Marfan Aorta

Frontiers in Cardiovascular Medicine, 2021
Medial deterioration leading to thoracic aortic aneurysms arises from multiple causes, chief among them mutations to the gene that encodes fibrillin-1 and leads to Marfan syndrome.
Cristina Cavinato, Minghao Chen, Dar Weiss, Maria Jesús Ruiz-Rodríguez, Martin A. Schwartz, Martin A. Schwartz, Jay D. Humphrey, Jay D. Humphrey   +7 more
doaj   +1 more source

Genetic testing in Marfan syndrome [PDF]

, 2016
Genetic testing is aiding rapid diagnosis of Marfan syndrome as a basis for management of eye, heart and skeletal disease. The affected patient's mutation can be used as a basis for prenatal or postnatal diagnosis of offspring.
Aragon-Martin, JA, Child, AH, Sage, K
core   +1 more source

Angiotensin II blockade and aortic-root dilation in Marfan's syndrome [PDF]

, 2008
Background: Progressive enlargement of the aortic root, leading to dissection, is the main cause of premature death in patients with Marfan's syndrome. Recent data from mouse models of Marfan's syndrome suggest that aortic-root enlargement is caused by ...
Brooke, Benjamin S., Dietz, Harry C., Habashi, Jennifer P., Judge, Daniel P., Loeys, Bart, Patel, Nishant   +5 more
core   +2 more sources

An iPSC-derived vascular model of Marfan syndrome identifies key mediators of smooth muscle cell death. [PDF]

, 2016
Marfan syndrome (MFS) is a heritable connective tissue disorder caused by mutations in FBN1, which encodes the extracellular matrix protein fibrillin-1.
Bernard, William George, Granata, Alessandra, Low, Lucinda, McNamara, Madeline, Sastry, Priya, Serrano, Felipe, Sinha, Sanjay   +6 more
core   +1 more source

Higher blood pressure in elderly hypertensive females, with increased arterial stiffness and blood pressure in females with the Fibrillin-1 2/3 genotype

BMC Cardiovascular Disorders, 2020
Background Elderly patients have a relatively high cardiovascular risk due to increased arterial stiffness, elevated blood pressure and decreased amounts of elastin in the arteries.
Ida Åström Malm, Urban Alehagen, Peter Blomstrand, Ulf Dahlström, Rachel De Basso   +4 more
doaj   +1 more source

The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin motifs) family [PDF]

, 2015
The ADAMTS (A Disintegrin and Metalloproteinase with Thrombospondin motifs) enzymes are secreted, multi-domain matrix-associated zinc metalloendopeptidases that have diverse roles in tissue morphogenesis and patho-physiological remodeling, in ...
A Colige, A Didangelos, A Luque, A Moncada-Pazos, A Noel, A Tan Ide, AC Jonsson-Rylander, AC Nicholson, AJ Fosang, B Joe, BH Koo, BH Koo, BV Nusgens, C Casal, C Esselens, C Gendron, C Kintakas, C Lopez-Otin, C Ricciardelli, CB Kern, CG Viloria, CH Mjaatvedt, CJ Liu, CJ Liu, CL Jacobi, CM Dancevic, CN Brocker, CR Flannery, D Wagsater, DL Russell, DL Silver, DR Edwards, DR McCulloch, DT Jones, Dylan R Edwards, EA Lin, EC Arner, ED Karagiannis, EM Majerus, F Guo, F Vazquez, FG Brunet, FX Gomis-Ruth, FX Gomis-Ruth, G Gao, G Gao, G Hashimoto, G Murphy, GC Choi, GE Lind, GG Levy, GJ Wayne, Grant N Wheeler, H Enomoto, H Jin, H Stanton, H Stanton, HL Lung, HM Brown, I Abbaszade, I Peluso, Ines Desanlis, J Dubail, J Dubail, J Felsenstein, J Hofsteenge, J Huxley-Jones, J Huxley-Jones, J Morales, JA Pyun, JC Adams, JC Rodriguez-Manzaneque, JC Rodriguez-Manzaneque, JC Rodriguez-Manzaneque, JD Sandy, JM Longpre, K Demircan, K Fujikawa, K Gopalakrishnan, K Kuno, K Kuno, K Stankunas, K Tamura, K Yamamoto, K Yamamoto, L Angerer, L Faivre, L Mittaz, L Mosyak, L Troeberg, L Troeberg, L Troeberg, L Wagstaff, LA Collins-Racie, LE Dupuis, LM Ricketts, M Cudic, M Hour El, M Kashiwagi, M Krampert, M Llamazares, MA Aldahmesh, MD Tortorella, MN Vankemmelbeke, N Dagoneau, N Hattori, N Rocks, N Rocks, N Stupka, NH Lim, NV Lee, NV Lee, P Wang, PH Lo, PJ Koshy, PS Chockalingam, R Kelwick, RC Salter, RH Song, Richard Kelwick, RL Robker, RP Somerville, RP Somerville, RS Patel, S Abdul-Majeed, S Gerhardt, S Kumar, S Kumar, S Nandadasa, S Porter, S Porter, SS Apte, SS Glasson, T Fontanil, T Shindo, TN Wight, U Auf Dem Keller, W Du, W Zeng, WE Kutz, WM Wang, X Lu, X Pu, XL Zheng, YJ Liu, YP Hsu   +145 more
core   +1 more source

"Computational Analysis of the Effect of fbn1 Gene Mutations in the Marfan Syndrome" [PDF]

Iranian Journal of Public Health, 2004
Fibrillin is a large glycoprotein synthesized in the tissues involved in Marfan syndrome, and known to be involved in tissue elasticity. The syndrome is corresponded to fbn1 gene and is characterized by cardiovascular, ocular, and skeletal abnormalities.
H Mohabatkar
doaj   +2 more sources

The role of epidermal growth factor-like module containing mucin-like hormone receptor 2 in human cancers. [PDF]

, 2014
G-protein coupled receptors (GPCRs) are among the most diverse and ubiquitous proteins in all of biology. The epidermal growth factor-seven span transmembrane (EGF-TM7) subfamily of adhesion GPCRs is a small subset whose members are mainly expressed on ...
Bloch, Orin, Ivan, Michael E, Kaur, Gurvinder, Oh, Michael C, Oh, Taemin, Parsa, Andrew T, Safaee, Michael, Sayegh, Eli T, Sun, Matthew Z   +8 more
core   +2 more sources

Delivery of sTRAIL variants by MSCs in combination with cytotoxic drug treatment leads to p53-independent enhanced antitumor effects [PDF]

, 2013
Mesenchymal stem cells (MSCs) are able to infiltrate tumor tissues and thereby effectively deliver gene therapeutic payloads. Here, we engineered murine MSCs (mMSCs) to express a secreted form of the TNF-related apoptosis-inducing ligand (TRAIL), which ...
A Ashkenazi, A Ashkenazi, A Chuntharapai, A Mohr, A Mohr, A Mohr, A Mohr, A Nakamizo, A Peister, AM van der Sloot, B Hall, C Buneker, CR Reis, D Sampath, D Stadel, DR Camidge, EW Duiker, FP Barry, GS Wu, GS Wu, I Nicoletti, J Lemke, J Luetzkendorf, JW Schymkowitz, K Ichikawa, K Papenfuss, L Zhang, LG Menon, LS Sasportas, M MacFarlane, M Vogler, M Vogler, M Vogler, ME Davis, MH Kim, MR Loebinger, MR Sprick, RM Zwacka, RW Johnstone, SB Bratton, SJ Braeuer, SM Albarenque, SM Kim, T Trarbach, TK Oost, TM Ganten, TS Griffith   +46 more
core   +1 more source