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
Aqueous humor TGFβ and fibrillin-1 in Tsk mice reveal clues to POAG pathogenesis [PDF]
Scientific ReportsAqueous humor (AH) and blood levels of transforming growth factor β (TGFβ) are elevated in idiopathic primary open angle glaucoma (POAG) representing a disease biomarker of unclear status and function.
James C. Tan +4 more
doaj +2 more sources
Fibrillin-1 regulates periostin expression during maintenance of periodontal homeostasis [PDF]
Journal of Dental Sciences, 2022 Background/purpose: Human periodontal ligament consists of elastic system fibers, mainly fibrillin-1 (FBN1). Periostin (POSTN) maintains periodontal homeostasis.
Yoshikazu Manabe +7 more
doaj +2 more sources
Cooperative Mechanism of ADAMTS/ ADAMTSL and Fibrillin-1 in the Marfan Syndrome and Acromelic Dysplasias [PDF]
Frontiers in Genetics, 2021 The term “fibrillinopathies” gathers various diseases with a wide spectrum of clinical features and severity but all share mutations in the fibrillin genes.
Pauline Arnaud +5 more
doaj +2 more sources
The Multiple Functions of Fibrillin-1 Microfibrils in Organismal Physiology. [PDF]
Int J Mol Sci, 2022 Fibrillin-1 is the major structural component of the 10 nm-diameter microfibrils that confer key physical and mechanical properties to virtually every tissue, alone and together with elastin in the elastic fibers. Mutations in fibrillin-1 cause pleiotropic manifestations in Marfan syndrome (MFS), including dissecting thoracic aortic aneurysms ...
Asano K +3 more
europepmc +4 more sources
In Vivo Studies of Mutant Fibrillin-1 Microfibrils [PDF]
Journal of Biological Chemistry, 2010 In humans, mutations in fibrillin-1 result in a variety of genetic disorders with distinct clinical phenotypes. While most of the known mutations in fibrillin-1 cause Marfan syndrome, a number of other mutations lead to clinical features unrelated to Marfan syndrome. Pathogenesis of Marfan syndrome is currently thought to be driven by mechanisms due to
Francesco Ramirez +2 more
exaly +3 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
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