Results 131 to 140 of about 18,239 (220)
Studies on TGF-β family signalling in the diseased lung tissue [PDF]
, 2012 This thesis focuses on transforming growth factor (TGF)-β signalling system in the human lung. Two lung parenchymal diseases, idiopathic pulmonary fibrosis (IPF) and chronic obstructive pulmonary disease (COPD), were studied.
Leppäranta, Outi
core
New insights into the structural role of EMILINs within the human skin microenvironment
Scientific ReportsSupramolecular extracellular matrix (ECM) networks play an essential role in skin architecture and function. Elastin microfibril interface-located proteins (EMILINs) comprise a family of three extracellular glycoproteins that serve as essential ...
Alvise Schiavinato +15 more
doaj +1 more source
Marfan syndrome: current perspectives
The Application of Clinical Genetics, 2016 Guglielmina Pepe,1,2 Betti Giusti,1,2 Elena Sticchi,1,2 Rosanna Abbate,1,2 Gian Franco Gensini,1–3 Stefano Nistri2,4 1Department of Experimental and Clinical Medicine, Section of Critical Medical Care and Medical Specialities, DENOTHE Center ...
Pepe G +5 more
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Journal of Immunology Research, 2016 Background. The clinical and histopathologic classification of anetoderma are not well characterized. Objective. We aimed to investigate the clinical and histopathologic characteristics of anetoderma and to correlate clinical phenotypes with ...
Jung Eun Kim +11 more
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The Biomechanics of Fibrillin Microfibrils: Lessons from the Ciliary Zonule
CellsMarfan 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 +6 more
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Fibrillin-1 Interactions with Heparin [PDF]
Journal of Biological Chemistry, 2005 Stuart A. Cain +7 more
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Exploring thoracic aorta ECM alterations in Marfan syndrome: insights into aorta wall structure
Scientific ReportsMarfan syndrome is a connective tissue disorder caused by FBN1 mutations, leading to aortic wall fragility and increased susceptibility to aneurysm and dissection. This study investigated microstructural and molecular alterations in the thoracic aorta of
Rodrigo Barbosa de Souza +15 more
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Zdravniški Vestnik, 2005 Background: The Marfan syndrome is an autosomal dominant heritable disorder of connective tissue. It is caused by mutations in the fibrillin-1 gene encoding glycoprotein fibrillin-1, a component of microfibrils of extracellular matrix.
Galina Pungerčič
doaj
Nature CommunicationsMicrofibril-associated glycoprotein 4 (MFAP4) is a 36-kDa extracellular matrix glycoprotein with critical roles in organ fibrosis, chronic obstructive pulmonary disease, and cardiovascular disorders, including aortic aneurysms.
Michael R. Wozny +6 more
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Acromicric dysplasia caused by a mutation of fibrillin 1 in a family: A case report. [PDF]
World J Clin Cases, 2023 Shen R, Feng JH, Yang SP.
europepmc +1 more source