Results 31 to 40 of about 22,293 (293)

Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development

bioRxiv, 2021
The extracellular matrix (ECM) undergoes an orchestrated transition from embryonic to mature ECM that is essential for postnatal life, yet the developmental transition mechanisms for ECM components and macromolecular complexes are poorly defined ...
T. Mead, Daniel R. Martin, Lauren W. Wang, S. Cain, Cagri Gulec, Elisabeth Cahill, Joseph C. Mauch, D. Reinhardt, C. Lo, C. Baldock, S. Apte   +10 more
semanticscholar   +1 more source

Fibrillin: from microfibril assembly to biomechanical function [PDF]

Philosophical Transactions of the Royal Society of London. Series B: Biological Sciences, 2002
Fibrillins form the structural framework of a unique and essential class of extracellular microfibrils that endow dynamic connective tissues with long–range elasticity. Their biological importance is emphasized by the linkage of fibrillin mutations to Marfan syndrome and related connective tissue disorders, which are associated with severe ...
Cay M. Kielty, Clair Baldock, David M. Lee, Matthew J. Rock, Jane Ashworth, C. Adrian Shuttleworth   +5 more
openalex   +9 more sources

LTBP1 promotes fibrillin incorporation into the extracellular matrix

bioRxiv, 2021
LTBP1 is a large extracellular matrix protein and an associated ligand of fibrillinmicrofibrils. Knowledge of LTBP1 functions is largely limited to its role in targeting and sequestering TGFβ growth factors within the extracellular matrix, thereby ...
Matthias Przyklenk, Veronika S. Georgieva, Fabian Metzen, S. Mostert, B. Kobbe, B. Callewaert, G. Sengle, B. Brachvogel, R. Mecham, M. Paulsson, R. Wagener, M. Koch, A. Schiavinato   +12 more
semanticscholar   +1 more source

Assembly of Epithelial Cell Fibrillins [PDF]

Journal of Investigative Dermatology, 2001
Fibrillins are large structural macromolecules that are components of connective tissue microfibrils. Fibrillin microfibrils have been found in association with basement membranes, where microfibrils appear to insert directly into the lamina densa.
Nevena Karaman-Jurukovska, Bette J. Dzamba, Lynn Y. Sakai, Zenzo Isogai, Marcia Simon, Noe L. Charbonneau, Douglas R. Keene   +6 more
openaire   +3 more sources

A Pan-Transcriptome Analysis Indicates Efficient Downregulation of the FIB Genes Plays a Critical Role in the Response of Alfalfa to Cold Stress

Plants, 2022
Alfalfa (Medicago sativa L.) is a perennial forage legume that is widely distributed throughout the world, and cold stress is an important environmental factor limiting the growth and production of alfalfa in cold regions. However, little is known of the
Xueqi Zhang, Huanhuan Yang, Manman Li, Yan Bai, Chao Chen, Donglin Guo, Changhong Guo, Yongjun Shu   +7 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

Altered Ocular Fibrillin Microfibril Composition in Mice With a Glaucoma-Causing Mutation of Adamts10

Investigative Ophthalmology and Visual Science, 2021
Purpose Previously, we identified a G661R mutation of ADAMTS10 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif 10) as being disease causative in a colony of Beagles with inherited primary open-angle glaucoma (POAG).
Hang-Jing Wu, D. Mortlock, Rachel W Kuchtey, J. Kuchtey   +3 more
semanticscholar   +1 more source

Autosomal Recessive Cutis Laxa 1C Mutations Disrupt the Structure and Interactions of Latent TGFβ Binding Protein-4

Frontiers in Genetics, 2021
Latent TGFβ binding protein-4 (LTBP4) is a multi-domain glycoprotein, essential for regulating the extracellular bioavailability of TGFβ and assembly of elastic fibre proteins, fibrillin-1 and tropoelastin.
Yasmene F. Alanazi, Michael P. Lockhart-Cairns, Stuart A. Cain, Thomas A. Jowitt, Anthony S. Weiss, Anthony S. Weiss, Anthony S. Weiss, Clair Baldock   +7 more
doaj   +1 more source

Contrast-Enhanced Tissue Processing of Fibrillin-Rich Elastic Fibres for 3D Visualization by Volume Scanning Electron Microscopy

Methods and Protocols, 2021
Elastic fibres constitute an important component of the extracellular matrix and currently are the subject of intensive study in order to elucidate their assembly, function and involvement in cell–matrix interactions and disease.
Philip N. Lewis, Robert D. Young, R. B. Souza, Andrew J. Quantock, Keith M. Meek   +4 more
doaj   +1 more source

A tandem duplication within the fibrillin 1 gene is associated with the mouse tight skin mutation. [PDF]

, 1996
Mice carrying the Tight skin (Tsk) mutation have thickened skin and visceral fibrosis resulting from an accumulation of extracellular matrix molecules.
Buchberg, Arthur M., Christner, Paul J., Jimenez, Sergio A., Ma, Qing, Manne, Jayanthi, McGrath, Rodney, Moskow, John J., Siracusa, Linda D.   +7 more
core   +3 more sources