Results 1 to 10 of about 9,961 (164)

The Diversity of Fibrillin Functions: Lessons from the Periodontal Ligament [PDF]

Cells
Marfan syndrome is caused by a mutation in the FBN1 gene encoding fibrillin-1. This extracellular matrix glycoprotein, which assembles into microfibrils, is best known for its scaffolding role in the production of elastic fibers responsible for ...
Elisabeth Genot, Tala Al Tabosh, Sylvain Catros, Florian Alonso, Damien Le Nihouannen   +4 more
doaj   +2 more sources

Arabidopsis thaliana Fibrillin 3a binds saturated fatty acids of chloroplast membranes [PDF]

Plant Signaling & Behavior
Fibrillins (FBNs) are conserved plastid lipid–associated proteins involved in lipid storage, stress adaptation and plastid ultrastructure. While several Arabidopsis thaliana FBNs have been functionally characterized, the biochemical properties of the ...
Andrés Herrera-Tequia, Kiran-Kumar Shivaiah, Peter Knut Lundquist   +2 more
doaj   +2 more sources

The Biomechanics of Fibrillin Microfibrils: Lessons from the Ciliary Zonule [PDF]

Cells
Marfan syndrome is an inherited connective tissue disorder that affects the cardiovascular, musculoskeletal, and ocular systems. It is caused by pathogenic variants in the fibrillin-1 gene (FBN1).
Pooja Rathaur, Juan Rodriguez, John Kuchtey, Samuel Insignares, Wendell B. Jones, Rachel W. Kuchtey, Steven Bassnett   +6 more
doaj   +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, N Taye, SZ Karoulias, D Hubmacher   +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, Daniel R Martin, Lauren W Wang, Stuart A Cain, Cagri Gulec, Elisabeth Cahill, Joseph Mauch, Dieter Reinhardt, Cecilia Lo, Clair Baldock, Suneel S Apte   +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, Megan Rushkin, BA, Derek Darby, BA, Trisha Chau, BS, Renee L. Shirley, PhD, Jeff S. King, PhD, Khanh Nguyen, MD, Gregory J. Landry, MD, Gregory L. Moneta, MD, Cherrie Abraham, MD, Lynn Y. Sakai, PhD, Amir F. Azarbal, MD   +11 more
doaj   +1 more source

Fibrillin-1 Regulates Arteriole Integrity in the Retina

Biomolecules, 2022
Fibrillin-1 is an extracellular matrix protein that assembles into microfibrils that provide critical functions in large blood vessels and other tissues.
Florian Alonso, Ling Li, Isabelle Fremaux, Dieter Peter Reinhardt, Elisabeth Génot   +4 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, Eylem Unlubilgin, Fatih Hizli, Muradiye Acar, Zeynep Kamalak, Aydin Kosus, Nermin Kosus, Deniz Hizli, Esra Gunduz   +8 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

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, Marie Saghaeian Jazi, Seyyed Mostafa Mir, Seyyed Mehdi Jafari   +3 more
doaj   +1 more source