Microfibril-associated Glycoprotein-2 (MAGP-2) Is Specifically Associated with Fibrillin-containing Microfibrils but Exhibits More Restricted Patterns of Tissue Localization and Developmental Expression Than Its Structural Relative MAGP-1 [PDF]
, 1998 Mark A. Gibson +3 more
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Aortic aneurysm and non-Hodgkin’s lymphoma in Marfan syndrome [PDF]
, 2009 The combination of Marfan syndrome with lymphoma is extremely rare. This report describes a case of Marfan syndrome who presented with chest discomfort and was diagnosed to have an aortic aneurysm and an additional incidental mediastinal mass that on ...
Sandip Kumar Ghosh +2 more
core
Structure of the Mutant Fibrillin-1 Gene in the Tight Skin (TSK) Mouse [PDF]
, 1997 C Bona
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Role of the Latent Transforming Growth Factor β–Binding Protein 1 in Fibrillin-Containing Microfibrils in Bone Cells In Vitro and In Vivo [PDF]
, 2000 Sarah L. Dallas +6 more
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The Microfibrillar Proteins MAGP-1 and Fibrillin-1 Form a Ternary Complex with the Chondroitin Sulfate Proteoglycan Decorin [PDF]
, 2000 Barbara Crippes Trask +3 more
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Mutations in Calcium-binding Epidermal Growth Factor Modules Render Fibrillin-1 Susceptible to Proteolysis [PDF]
, 2000 Dieter P. Reinhardt +5 more
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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
Induction of Skin Fibrosis in Mice Expressing a Mutated Fibrillin-1 Gene [PDF]
, 2000 Shinichiro Saito +6 more
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Proteomic fingerprints of damage in extracellular matrix assemblies
Matrix Biology Plus, 2020 In contrast to the dynamic intracellular environment, structural extracellular matrix (ECM) proteins with half-lives measured in decades, are susceptible to accumulating damage.
Alexander Eckersley +9 more
doaj
Fluid dynamics of aortic root dilation in Marfan syndrome
, 2014 Aortic root dilation and propensity to dissection are typical manifestations of the Marfan Syndrome (MS), a genetic defect leading to the degeneration of the elastic fibres. Dilation affects the structure of the flow and, in turn, altered flow may play a
Costantini, Martina +4 more
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