Results 51 to 60 of about 22,293 (293)

Circulating fibrillin fragment concentrations in patients with and without aortic pathology

JVS - Vascular Science, 2022
Objective: Fragments of fibrillin-1 and fibrillin-2 will be detectable in the plasma of patients with aortic dissections and aneurysms. We sought to determine whether the plasma fibrillin fragment levels (PFFLs) differ between patients with thoracic ...
Eric J. Carlson, PhD, Megan Rushkin, BA, Derek Darby, BA, Trisha Chau, BS, Renee L. Shirley, PhD, Jeff S. King, PhD, Khanh Nguyen, MD, Gregory J. Landry, MD, Gregory L. Moneta, MD, Cherrie Abraham, MD, Lynn Y. Sakai, PhD, Amir F. Azarbal, MD   +11 more
doaj  

Case report: Biochemical and clinical phenotypes caused by cysteine substitutions in the epidermal growth factor-like domains of fibrillin-1

Frontiers in Genetics, 2022
Marfan syndrome, an autosomal dominant disorder of connective tissue, is primarily caused by mutations in the fibrillin-1 (FBN1) gene, which encodes the protein fibrillin-1.
Xin Liu, Kaiqing Liu, Danyao Nie, Jing Zhang, Liyun Zhang, Xinhua Liu, Jiantao Wang   +6 more
doaj   +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

Fibrillin-Rich Microfibrils are Reduced in Photoaged Skin. Distribution at the Dermal–Epidermal Junction [PDF]

, 1999
Chronic sun exposure results in photoaged skin with deep coarse wrinkles and loss of elasticity. We have examined the distribution and abundance of fibrillin-rich microfibrils, key structural components of the elastic fiber network, in photoaged and ...
Craven, Nicholas M., Griffiths, Christopher E.M., Kielty, Cay M., Shuttleworth, C. Adrian, Watson, Rachel E.B.   +4 more
core   +1 more source

Interaction of Tropoelastin with the Amino-terminal Domains of Fibrillin-1 and Fibrillin-2 Suggests a Role for the Fibrillins in Elastic Fiber Assembly [PDF]

Journal of Biological Chemistry, 2000
Alignment of tropoelastin molecules during the process of elastogenesis is thought to require fibrillin-containing microfibrils. In this study, we have demonstrated that amino-terminal domains of two microfibrillar proteins, fibrillin-1 and fibrillin-2, interact with tropoelastin in solid phase binding assays.
Timothy M. Ritty, Barbara Crippes Trask, Robert P. Mecham, William R. Abrams, Joel Rosenbloom, Timothy M. Trask   +5 more
openaire   +3 more sources

Late spontaneous bilateral intraocular lens subluxation accompanied with intraocular pressure elevation in a patient with acromegaly

Taiwan Journal of Ophthalmology, 2020
A 53-year-old male with newly diagnosed acromegaly came to our clinic with the chief complaint of diplopia. He had the past ocular history of uneventful phacoemulsification cataract surgery with intraocular lens (IOL) implantation in the right eye 17 ...
Yin-Hsi Chang, San-Ni Chen
doaj   +1 more source

Infection of the brown alga Ectocarpus siliculosus by the oomycete Eurychasma dicksonii induces oxidative stress and halogen metabolism [PDF]

, 2016
Acknowledgments We would like to thank the Aberdeen Proteome Facility, especially Phil Cash, David Stead and Evelyn Argo for assistance with 2D electrophoresis and mass spectrometry. M.S.
Asada, Austin, Bhadauria, Bouarab, Bréhélin, Butler, Chen, Chen, Cock, Cock, Collén, Contreras, Cosse, Crépineau, Dantuma, Dittami, Farmer, Farnham, Fosu-Nyarko, Franco, Gachon, Gachon, Goulitquer, Grenville-Briggs, Grenville-Briggs, Grenville-Briggs, Grenville-Briggs, Gschloessl, Heesch, Kav, Klochkova, Konotchick, Küpper, Küpper, Küpper, Küpper, Küpper, Küpper, Küpper, Küpper, Küpper, La Barre, Langenkämper, Le Bail, Matros, Mayer, Mithöfer, Müller, Nelson, Orozco-Cárdenas, Palmer, Park, Paul, Pearson, Pfaffl, Potin, Ritter, Roeder, Roy, Salgado, Santino, Schmidt, Sekimoto, Singh, Strittmatter, Strittmatter, Tan, Thomas, Thomas, Tonon, Tsirigoti, Tsirigoti, Uppalapati, Vidi, Vizcaíno, Weinberger, Weinberger, Weinberger, West, West, Yang, Youssef, Ytterberg, Zambounis, Zhou   +84 more
core   +1 more source

Chromoplast biogenesis in Chelidonium majus petals

Acta Societatis Botanicorum Poloniae, 2011
The differentiation of chromoplasts, with special emphasis on the formation and the organisation of chromoplast fibrils, was followed in the petals of the greater celandine, Chelidonium majus L.
Nikola Ljubešić, Mercedes Wrischer
doaj   +1 more source

Fibrillin-1 mutations causing Weill-Marchesani syndrome and acromicric and geleophysic dysplasias disrupt heparan sulfate interactions. [PDF]

PLoS ONE, 2012
The extracellular glycoprotein fibrillin-1 forms microfibrils that act as the template for elastic fibers. Most mutations in fibrillin-1 cause Marfan syndrome with severe cardiovascular and ocular symptoms, and tall stature.
Stuart A Cain, Amanda McGovern, Andrew K Baldwin, Clair Baldock, Cay M Kielty   +4 more
doaj   +1 more source

Structure of the Integrin Binding Fragment from Fibrillin-1 Gives New Insights into Microfibril Organization [PDF]

, 2004
Human fibrillin-1, the major structural protein of extracellular matrix (ECM) 10–12 nm microfibrils, is dominated by 43 calcium binding epidermal growth factor-like (cbEGF) and 7 transforming growth factor β binding protein-like (TB) domains.
Altroff, Altroff, Arnaout, Baldock, Bax, Brunger, Cardy, CCP4, Chobotova, Colman, Dallas, de La Fortelle, Dietz, Downing, Eriksen, Esnouf, Fowler, Garcia, Gibson, Gibson, Giltay, Gouet, Handford, Harlos, Hotchin, Irie, Isogai, Jensen, Kabsch, Kanzaki, Kettle, Kielty, Knott, Krawczak, Laskowski, Lawrence, Lin, Lin, Logan, Mardon, McGettrick, Merritt, Moren, Mould, Obara, Oberhauser, Otwinowski, Pereira, Pfaff, Qian, Rao, Reinhardt, Reinhardt, Reinhardt, Saharinen, Sakai, Sakai, Sakamoto, Sheldrick, Sherratt, Sherratt, Stuart, Taipale, Takagi, Thompson, Trask, Werner, Wess, Xiong, Xiong, Yin, Yuan, Yuan, Zhang, Zhang   +74 more
core   +3 more sources