Abnormal Activation of BMP Signaling Causes Myopathy in Fbn2 Null Mice.
Fibrillins are large extracellular macromolecules that polymerize to form the backbone structure of connective tissue microfibrils. Mutations in the gene for fibrillin-1 cause the Marfan syndrome, while mutations in the gene for fibrillin-2 cause ...
Gerhard Sengle +8 more
doaj +1 more source
Live Imaging of Type I Collagen Assembly Dynamics in Osteoblasts Stably Expressing GFP and mCherry-Tagged Collagen Constructs [PDF]
Type I collagen is the most abundant extracellular matrix protein in bone and other connective tissues and plays key roles in normal and pathological bone formation as well as in connective tissue disorders and fibrosis.
Bonewald, Lynda F. +9 more
core +2 more sources
Irreversible ECM proteolysis by remodeling enzymes shapes development, homeostasis, and disease. ECM‐degrading proteases display cell specificity and are governed by shared mechanisms, exhibiting functional redundancy in generating matrikines, growth factors, and cytokines.
Inna Solomonov, Orit Kollet, Irit Sagi
wiley +1 more source
Microenvironmental Regulation by Fibrillin-1
Fibrillin-1 is a ubiquitous extracellular matrix molecule that sequesters latent growth factor complexes. A role for fibrillin-1 in specifying tissue microenvironments has not been elucidated, even though the concept that fibrillin-1 provides extracellular control of growth factor signaling is currently appreciated.
Sengle, Gerhard +14 more
openaire +5 more sources
Congenital ectopia lentis : diagnosis and treatment [PDF]
Congenital ectopia lentis (EL) is an ocular condition, which typically causes a high grade of refractive errors, mainly myopia and astigmatism. These might be difficult to compensate for, especially in children, who might develop ametropic amblyopia ...
Rysä Konradsen, Tiina
core +1 more source
A guide to the types, structures, and multifaceted functions of matrix metalloproteinases in cancer
Matrix metalloproteinases (MMPs) orchestrate cancer progression and metastasis through proteolytic and non‐proteolytic actions. By remodeling the tumor microenvironment, enhancing growth factor availability, and modulating cell behavior, MMPs promote proliferation, migration or invasion, and epithelial‐to‐mesenchymal transition. Alongside extracellular
Zoi Piperigkou +4 more
wiley +1 more source
Fibrillin: from microfibril assembly to biomechanical function [PDF]
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 ...
Baldock, C +5 more
openaire +6 more sources
Studies on TGF-β family signalling in the diseased lung tissue [PDF]
This thesis focuses on transforming growth factor (TGF)-β signalling system in the human lung. Two lung parenchymal diseases, idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD), were studied.
Leppäranta, Outi
core
MAGP2 Controls Notch via Interactions with RGD Binding Integrins: Identification of a Novel ECM-Integrin-Notch Signaling Axis [PDF]
Canonical Notch signaling involves Notch receptor activation via interaction with cell surface bound Notch ligand. Recent findings also indicate that Notch signaling may be modulated by cross-talk with other signaling mechanisms.
Albig, Allan R. +6 more
core +2 more sources
Behind the scenes: how the EMILIN/Multimerin family shapes the cancer landscape
The EMILIN/Multimerin family members regulate key hallmarks of cancer—including apoptosis, angiogenesis, metastasis, and tumor microenvironment remodeling. As indicated, their function in immune evasion, drug resistance, and metabolic reprogramming remains largely unexplored.
Evelina Poletto +9 more
wiley +1 more source

