Fibrillin-1 regulates endothelial sprouting during angiogenesis. [PDF]
Proc Natl Acad Sci U S A, 2023 Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils which provide critical functions in large blood vessels and other tissues. Mutations in the fibrillin-1 gene are associated with cardiovascular, ocular, and skeletal abnormalities in Marfan syndrome. Here, we reveal that fibrillin-1 is critical for angiogenesis
Alonso F +7 more
europepmc +3 more sources
Succinylation Inhibits the Enzymatic Hydrolysis of the Extracellular Matrix Protein Fibrillin 1 and Promotes Gastric Cancer Progression [PDF]
Advanced Science, 2022 exaly +2 more sources
Macromolecular crowding enhances fibrillin-1 deposition in the extracellular matrix [PDF]
European Cells & Materials, 2022 Biochemical and biophysical factors need consideration when modelling in vivo cellular behaviour using in vitro cell culture systems. One underappreciated factor is the high concentration of macromolecules present in vivo, which is typically not ...
B Satz-Jacobowitz +3 more
doaj +1 more source
Fibrillin-1 and fibrillin-1-derived asprosin in adipose tissue function and metabolic disorders. [PDF]
J Cell Commun Signal, 2020 The extracellular matrix microenvironment of adipose tissue is of critical importance for the differentiation, remodeling and function of adipocytes. Fibrillin-1 is one of the main components of microfibrils and a key player in this process. Furin processing of profibrillin-1 results in mature fibrillin-1 and releases the C-terminal propeptide as a ...
Muthu ML, Reinhardt DP.
europepmc +4 more sources
Fibrillin-1–enriched microenvironment drives endothelial injury and vascular rarefaction in chronic kidney disease [PDF]
Science Advances, 2021 Li Li, Jinlin Liao, Qian Yuan
exaly +2 more sources
Proteolysis of fibrillin-2 microfibrils is essential for normal skeletal development
eLife, 2022 The embryonic extracellular matrix (ECM) undergoes transition to mature ECM as development progresses, yet few mechanisms ensuring ECM proteostasis during this period are known.
Timothy J Mead +10 more
doaj +1 more source
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 +11 more
doaj +1 more source
Is There a Relationship Between Pelvic Organ Prolapse and Tissue Fibrillin-1 Levels? [PDF]
International Neurourology Journal, 2015 Purpose: Pelvic organ prolapse is a multifactorial disorder in which extracellular matrix defects are implicated. Fibrillin-1 level is reduced in stress urinary incontinence.
Ayla Eser +8 more
doaj +1 more source
Role of fibrilins in human cancer: A narrative review
Health Science Reports, 2023 Background Fibrillin is one of the extracellular matrix glycoproteins and participates in forming microfibrils found in many connective tissues. The microfibrils enable the elasticity and stretching properties of the ligaments and support connective ...
Mahsa Mahdizadehi +3 more
doaj +1 more source
POGLUT2 and POGLUT3 O-glucosylate multiple EGF repeats in fibrillin-1, -2, and LTBP1 and promote secretion of fibrillin-1. [PDF]
J Biol Chem, 2021 Fibrillin-1 (FBN1) is the major component of extracellular matrix microfibrils, which are required for proper development of elastic tissues, including the heart and lungs. Through protein-protein interactions with latent transforming growth factor (TGF) β-binding protein 1 (LTBP1), microfibrils regulate TGF-β signaling.
Williamson DB +3 more
europepmc +4 more sources