Fibrillin-1 and fibrillin-1-derived asprosin in adipose tissue function and metabolic disorders [PDF]
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
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"Computational Analysis of the Effect of fbn1 Gene Mutations in the Marfan Syndrome" [PDF]
Fibrillin is a large glycoprotein synthesized in the tissues involved in Marfan syndrome, and known to be involved in tissue elasticity. The syndrome is corresponded to fbn1 gene and is characterized by cardiovascular, ocular, and skeletal abnormalities.
H Mohabatkar
doaj +1 more source
Secreted a disintegrin-like and metalloprotease with thrombospondin type 1 motif (ADAMTS) proteases play crucial roles in tissue development and homeostasis.
Stylianos Z. Karoulias+3 more
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A heart for fibrillin : spatial arrangement in adult wild-type murine myocardial tissue [PDF]
Fibrillins are major constituents of microfibrils, which are essential components of the extracellular matrix of connective tissues where they contribute to the tissue homeostasis.
De Backer, Julie+4 more
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Circulating fibrillin fragment concentrations in patients with and without aortic pathology
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
Angiotensin II blockade and aortic-root dilation in Marfan's syndrome [PDF]
Background: Progressive enlargement of the aortic root, leading to dissection, is the main cause of premature death in patients with Marfan's syndrome. Recent data from mouse models of Marfan's syndrome suggest that aortic-root enlargement is caused by ...
Brooke, Benjamin S.+5 more
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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
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Chronic tendon pathology: molecular basis and therapeutic implications [PDF]
Tendons are frequently affected by chronic pain or rupture. Many causative factors have been implicated in the pathology, which until relatively recently was under-researched and poorly understood.
Riley, GP
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Interaction of Tropoelastin with the Amino-terminal Domains of Fibrillin-1 and Fibrillin-2 Suggests a Role for the Fibrillins in Elastic Fiber Assembly [PDF]
Alignment of tropoelastin molecules during the process of elastogenesis is thought to require fibrillin-containing microfibrils. In this study, we have demonstrated that amino-terminal domains of two microfibrillar proteins, fibrillin-1 and fibrillin-2, interact with tropoelastin in solid phase binding assays.
Timothy M. Ritty+5 more
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Metabolic and molecular events occurring during chromoplast biogenesis [PDF]
Chromoplasts are nonphotosynthetic plastids that accumulate carotenoids. They derive from other plastid forms, mostly chloroplasts. The biochemical events responsible for the interconversion of one plastid form into another are poorly documented. However,
Barsan, Cristina+8 more
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