Results 61 to 70 of about 7,077 (251)
ENU mutagenesis reveals a novel phenotype of reduced limb strength in mice lacking fibrillin 2.
PLoS ONE, 2010 BackgroundFibrillins 1 (FBN1) and 2 (FBN2) are components of microfibrils, microfilaments that are present in many connective tissues, either alone or in association with elastin.
Gaynor Miller +13 more
doaj +1 more source
Fibulin-5 Contributes to Microfibril Assembly in Human Periodontal Ligament Cells [PDF]
, 2009 The elastic system fibers comprise oxytalan, elaunin and elastic fibers, which differ in their relative microfibril and elastin content. Human periodontal ligaments (PDL) contain only oxytalan fibers (pure microfibrils) among them.
Hatakeyama, Yuji +6 more
core +2 more sources
A patient with Marfan syndrome in a general practitioner’s office
Pediatria i Medycyna Rodzinna, 2015 Marfan syndrome is a disorder of the connective tissue (fibrillins and elastins). It is an autosomal dominant disease associated with a defect of chromosome 15 which encodes fibrillin-1.
Magdalena Czerżyńska +3 more
doaj +1 more source
The interfascicular matrix enables fascicle sliding and recovery in tendon, and behaves more elastically in energy storing tendons [PDF]
, 2015 While the predominant function of all tendons is to transfer force from muscle to bone and position the limbs, some tendons additionally function as energy stores, reducing the cost of locomotion.
Birch, Helen L. +5 more
core +5 more sources
Ocular Findings as the Most Striking Manifestation of a SMAD3 Variant
American Journal of Medical Genetics Part A, EarlyView.ABSTRACT Loeys‐Dietz syndrome (LDS) is a heritable connective tissue disorder with variable expressivity. It is a multisystemic condition mainly characterized by a propensity for arterial aneurysms and dissections, skeletal manifestations, hypertelorism, bifid uvula, craniosynostosis, and cutaneous features.
Noémie Villeneuve‐Cloutier +7 more
wiley +1 more source
Bone matrix to growth factors: location, location, location [PDF]
, 2010 The demonstration that fibrillin-1 mutations perturb transforming growth factor (TGF)–β bioavailability/signaling in Marfan syndrome (MFS) changed the view of the extracellular matrix as a passive structural support to a dynamic modulator of cell ...
Rifkin, Daniel B., Todorovic, Vesna
core +2 more sources
Arthritis &Rheumatology, EarlyView.Objective Systemic sclerosis (SSc) is a connective tissue disease with fibrotic remodeling of the skin and various internal organs. SSc is associated with the highest case‐specific mortality of all rheumatic autoimmune diseases with limited antifibrotic treatment options.
Thuong Trinh‐Minh +12 more
wiley +1 more source
Microscopy Research and Technique, EarlyView.The figure presents the trilaminar organization of the human deep fascia, composed of a thick, collagen‐rich intermediate layer flanked by thinner superficial and deep layers with loose connective tissue. ABSTRACT Traditional histological methods provide limited insights into the complex 3D microstructure of fascia and its relationship to disease. This
Chiedozie Kenneth Ugwoke +6 more
wiley +1 more source
Developmental Biology, 1995 A monoclonal antibody generated against the isolated extracellular matrix (ECM) of the medusa Podocoryne carnea M. Sars (Coelenterata, Cnidaria, Hydrozoa) stains a fibrillar component of the Podocoryne ECMs in immunohistochemical preparations.
S. Reber‐Müller +4 more
semanticscholar +1 more source
Experimental Dermatology, 2020 Supramolecular networks composed of fibrillins (fibrillin‐1 and fibrillin‐2) and associated ligands form intricate cellular microenvironments which balance skin homoeostasis and direct remodelling. Fibrillins assemble into microfibrils which are not only
Christin S. Adamo +2 more
semanticscholar +1 more source