Results 1 to 10 of about 371 (116)

Fibrillin-1 Regulates Arteriole Integrity in the Retina

open access: yesBiomolecules, 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   +4 more
doaj   +3 more sources

Microenvironmental regulation by fibrillin-1.

open access: yesPLoS Genetics, 2012
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 ...
Gerhard Sengle   +14 more
doaj   +4 more sources

Macromolecular crowding enhances fibrillin-1 deposition in the extracellular matrix [PDF]

open access: yesEuropean 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

open access: yeseLife, 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

Fibrillin-1 and fibrillin-1-derived asprosin in adipose tissue function and metabolic disorders [PDF]

open access: yesJournal of Cell Communication and Signaling, 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 L. Muthu, Dieter P. Reinhardt
openaire   +2 more sources

Circulating fibrillin fragment concentrations in patients with and without aortic pathology

open access: yesJVS - 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]

open access: yesInternational 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

open access: yesHealth 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

Case report: Biochemical and clinical phenotypes caused by cysteine substitutions in the epidermal growth factor-like domains of fibrillin-1

open access: yesFrontiers in Genetics, 2022
Marfan syndrome, an autosomal dominant disorder of connective tissue, is primarily caused by mutations in the fibrillin-1 (FBN1) gene, which encodes the protein fibrillin-1.
Xin Liu   +6 more
doaj   +1 more source

Homotypic Fibrillin-1 Interactions in Microfibril Assembly [PDF]

open access: yesJournal of Biological Chemistry, 2005
We have defined the homotypic interactions of fibrillin-1 to obtain new insights into microfibril assembly. Dose-dependent saturable high affinity binding was demonstrated between N-terminal fragments, between furin processed C-terminal fragments, and between these N- and C-terminal fragments.
Marson, Andrew   +8 more
openaire   +3 more sources

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