Extracellular Matrix
Chapters
« previous | page 2 of 2 pagesSilica-Induced Inflammatory Mediators and Pulmonary Fibrosis
Andrea K. Hubbard, Sarah Mowbray, Michael Thibodeau and Charles Giardina
Silicosis continues to be a lung disease with significant morbidity and mortality. Although silica- induced lung injury and cell activation and/or death has been investigated over the past several years, basic research continues to reveal the cell: cell and cell: mediator interactions critical to...
Surgery for Cardiovascular Disorders in Marfan Syndrome: The Atrioventricular Valves, Distal Aortic Segments and Myocardium
Roland Hetzer, Reinhard Pregla and Frank Barthel
The life expectancy of patients with Marfan syndrome is primarily limited by severe complications of the malformations of the aortic root and the ascending aorta root typical of the disease. The characteristic structural defects of the aortic wall give rise to acute type A dissection, i.e., disru...
The Fibrillins and Key Molecular Mechanisms that Initiate Disease Pathways
Lynn Y. Sakai
In 1986, fibrillin was first identified as a large noncollagenous glycoprotein associated with a certain type of microfibril ubiquitous in the connective tissue space.1 Because fibrillin monoclonal antibodies displayed a clear periodic labeling of microfibrils, we proposed that fibrillin was a ma...
The Marfan Mutation Database
Gwenaëlle Collod-Béroud and Catherine Boileau
Mutations in the fibrillin gene (FBN1) were described at first in Marfan syndrome (MFS) patients. Subsequently, the gene was shown to harbor mutations related to a spectrum of conditions clinically related to MFS, the «type-1 fibrillinopathies». In an effort to standardize the information regardi...
Tissue Scarring Lessons from Wound Healing
Mohammed S. Razzaque, Moussa El-Hallak, Abdallah Azouz and Takashi Taguchi
Tissue scarring due to abnormal matrix remodeling is an important cause of organ fail ure, which is a leading cause of morbidity and mortality. Current studies have focused on determination of the molecular basis of controlled wound healing, and uncontrolled tissue scarring. Scarless repair is a ...
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