Results 21 to 30 of about 6,142 (190)

Fibrillins Can Co-assemble in Fibrils, but Fibrillin Fibril Composition Displays Cell-specific Differences*

Journal of Biological Chemistry, 2003
Fibrillins are microfibril-forming extracellular matrix macromolecules that modulate skeletal development. In humans, mutations in fibrillins result in long bone overgrowth as well as other distinct phenotypes.
N. Charbonneau, B. Dzamba, R. Ono, D. Keene, G. Corson, D. Reinhardt, L. Sakai   +6 more
semanticscholar   +1 more source

ENU mutagenesis reveals a novel phenotype of reduced limb strength in mice lacking fibrillin 2.

PLoS ONE, 2010
BackgroundFibrillins 1 (FBN1) and 2 (FBN2) are components of microfibrils, microfilaments that are present in many connective tissues, either alone or in association with elastin.
Gaynor Miller, Monica Neilan, Ruth Chia, Nabeia Gheryani, Natalie Holt, Annabelle Charbit, Sara Wells, Valter Tucci, Zuzanne Lalanne, Paul Denny, Elizabeth M C Fisher, Michael Cheeseman, Graham N Askew, T Neil Dear   +13 more
doaj   +1 more source

MAGP-2 Has Multiple Binding Regions on Fibrillins and Has Covalent Periodic Association with Fibrillin-containing Microfibrils*

Journal of Biological Chemistry, 2004
The interactions of microfibril-associated glycoprotein (MAGP)-2 have been investigated with fibrillins and fibrillin-containing microfibrils. Solid phase binding assays were conducted with recombinant fragments covering fibrillin-1 and most of fibrillin-
E. Hanssen, F. H. Hew, E. Moore, M. Gibson   +3 more
semanticscholar   +1 more source

Fibulin-4 deposition requires EMILIN-1 in the extracellular matrix of osteoblasts

Scientific Reports, 2017
Tissue microenvironments formed by extracellular matrix networks play an important role in regulating tissue structure and function. Extracellular microfibrillar networks composed of fibrillins and their associated ligands such as LTBPs, fibulins, and ...
Alvise Schiavinato, Douglas R. Keene, Thomas Imhof, Roberto Doliana, Takako Sasaki, Gerhard Sengle   +5 more
doaj   +1 more source

Arabidopsis fibrillin 1-2 subfamily members exert their functions via specific protein–protein interactions

Journal of Experimental Botany, 2021
Fibrillins (FBNs) are plastidial proteins found in photosynthetic organisms from cyanobacteria to higher plants. The function of most FBNs remains unknown. Here, we focused on members of the FBN subgroup comprising FBN1a, FBN1b, and FBN2.
Diego Torres-Romero, Ángeles Gómez-Zambrano, A. Serrato, M. Sahrawy, Á. Mérida   +4 more
semanticscholar   +1 more source

Elastin in the Liver

Frontiers in Physiology, 2016
A characteristic feature of liver cirrhosis is the accumulation of large amounts of connective tissue with the prevailing content of type I collagen. Elastin is a minor connective tissue component in normal liver but it is actively synthesized by hepatic
Jiri Kanta
doaj   +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*

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 ...
Timothy M. Trask, B. Trask, T. Ritty, W. Abrams, J. Rosenbloom, R. Mecham   +5 more
semanticscholar   +1 more source

Putative functions of extracellular matrix glycoproteins in secondary palate morphogenesis

Frontiers in Physiology, 2012
Cleft palate is a common birth defect in humans. Elevation and fusion of paired palatal shelves are coordinated by growth and transcription factors, and mutations in these can cause malformations.
Rocca ed'Amaro, Rolf eScheidegger, Susan eBlumer, Pawel ePazera, Christos eKatsaros, Daniel eGraf, Matthias eChiquet   +6 more
doaj   +1 more source

A patient with Marfan syndrome in a general practitioner’s office

Pediatria i Medycyna Rodzinna, 2015
Marfan syndrome is a disorder of the connective tissue (fibrillins and elastins). It is an autosomal dominant disease associated with a defect of chromosome 15 which encodes fibrillin-1.
Magdalena Czerżyńska, Zuzanna Judyta Tyrakowska, Anna Wiśniewska, Emilia Rozwadowska   +3 more
doaj   +1 more source

IL‐4/STAT6‐signaling Influences Local Inflammation and Regeneration Processes During Acute Pancreatitis and Promotes Fibrosis by a Direct Activation of Pancreatic Fibroblasts During Chronic Pancreatitis

Advanced Science, EarlyView.
IL‐4/IL‐13/STAT6 signaling plays a crucial for the suppression of pro‐inflammation during acute pancreatitis and supports acinar cell regeneration but has only minor impact on fibrogenesis during chronic form of the disease. IL‐4/IL‐13 induce the expression of certain collagens directly in pancreatic fibroblasts via STAT6 activation, whereas ...
ElSheikh H, Salvers A, Piesker J, Buchholz S, Lange R, Ameling S, Steil L, Hammer E, Homuth G, Völker U, Bröker BM, Weiss FU, Sendler M, Glaubitz J   +13 more
wiley   +1 more source