Extracellular Matrix
Chapters
page 1 of 2 pages | next »Assembly of Microfibrils
Kerstin Tiedemann, Boris Bätge and Dieter P. Reinhardt
Fibrillins are physiologically secreted as extended thread-like monomers into the extracel- lular matrix by many cell types. The mature functional entity, however, is constituted by higher order aggregates which are called microfibrils.1 The assembly process of forming these supramolecular stru...
Can Manipulation of Apoptotic Cell Death Benefit Tissue Scarring?
Wesam Ahmed, Mohammed S. Razzaque and Takashi Taguchi
Cell death by apoptosis is an active process of cell removal that is initiated and regulated by activation of specific enzymes and signaling molecules. In contrast to necrotic cell death, apoptotic cell death holds the potential for therapeutic manipulation. Recent studies document important role...
Cardiac Fibrosis and Aging
Serge Masson, Roberto Latini, Monica Salio and Fabio Fiordaliso
Excessive deposition of extracellular matrix proteins during aging leads to a progressive reduction of myocardial and arterial compliances. The increased cardiovascular stiffness may in turn determine a reduced capacity of the aged heart to respond to stressful situations. Our knowledge on the bi...
Cardiovascular Aspects of the Marfan Syndrome—A Systematic Review
Yskert von Kodolitsch and Maike Rybczynski
The Marfan syndrome (MFS) is an autosomal dominant disorder of connective tissue caused by mutations of the fibrillin-1 gene, which codes for fibrillin, a major component of the extracellular microfibrils. The mean life expectancy in untreated MFS is 32 years with aortic dissection, aortic ruptur...
Cardiovascular Surgery (II): Surgical Management of the Marfan Patient at the Johns Hopkins Hospital
Duke E. Cameron and Vincent L. Gott
Over the past 25 years, there has been remarkable progress in surgical management of the Marfan patient with aortic root aneurysm. The introduction in 1966 of the composite graft procedure by Mr. Hugh Bentall in London was a major step forward in the successful treatment of these aneurysms and wa...
Cardiovascular Surgery: Surgical Management of the Marfan Patient at the Johns Hopkins Hospital
Duke E. Cameron and Vincent L. Gott
Over the past 25 years, there has been remarkable progress in surgical management of the Marfan patient with aortic root aneurysm. The introduction in 1966 of the composite graft procedure by Mr. Hugh Bentall in London was a major step forward in the successful treatment of these aneurysms and wa...
Diagnosis and Treatment of Marfan Syndrome—A Summary
Anne H. Child, Luitgard Neumann and Peter N. Robinson
Marfan syndrome (MFS) is a dominantly inherited disease of connective tissue with diverse manifestations (Table 1), involving primarily the skeletal, ocular, and cardiovascular systems. Affected individuals may have a characteristic habitus including tall stature and dolichostenomelia, arachnodac...
Familial Thoracic Aortic Aneurysms and Dissections
Sumera N. Hasham and Dianna M. Milewicz
Ascending aortic aneurysms leading to type A aortic dissections are the major cardiovascular complication of the Marfan syndrome (MFS). MFS is a major genetic syndrome predisposing individuals to these aortic conditions but other genetic syndromes also have similar aortic problems. In addition, a...
Genetics of Marfan Syndrome in Mouse Models
Emilio Arteaga-Solis, Harry Dietz and Francesco Ramirez
Tensile strength and resilience are critically important properties of the connective tissue that are afforded by the assembly of specialized collagenous and elastic fiber networks. In addition to conferring integrity to virtually every organ system, these extracellular macroaggregates are also i...
Historical Introduction: The Marfan Syndrome: From Clinical Delineation to Mutational Characterization, a Semiautobiographic Account
Victor A. McKusick
In 1876, E. Williams,1 an ophthalmologist in Cincinnati, Ohio, described ectopia lentis in a brother and sister who were exceptionally tall and had been loosejointed from birth. Although there is a Williams syndrome that has aortic manifestations (supravalvar aortic stenosis), the name William...
Insights into Fibrillin-1 Structure and Function from Domain Studies
Pat Whiteman and Penny A. Handford
Structural information is required to understand the assembly of fibrillin-1 into 10-12 nm microfibrils and to gain insight into the consequences of Marfan syndrome (MFS)-causing mutations. Since fibrillin-1 is a modular protein (Fig. 1), a dissection approach has been used to generate structural...
Involvement of NF-kB in Renal Inflammation and Sclerosis
Laurent Baud, Bruno Fouqueray, Agnes Bellocq, Jean-Philippe Haymannn and Julie Peltier
Nuclear factor-kB (NF-kB) comprises a family of transcription factors. They are thought to have a central role in the expression of genes involved in cell mobilization, cell proliferation and cell differentiation, and, hence, in inflammation, repair and fibrosis processes. In particular, NF-kB ac...
Low-Density Lipoprotein and Glomerulosclerosis
Hyun Soon Lee
Hypercholesterolemia is a common feature of nephrosis or uremia. Dietary hypercholesterolemia aggravates the renal injury in experimental focal segmental glomerulosclerosis (FSGS). Hypercholesterolemia is mainly due to increased level of low-density lipoprotein (LDL). Accumulation of apolipoprote...
Microfibril-Associated Glycoprotein-1 (MAGP-1) and Other Non-fibrillin Macromolecules Which May Possess a Functional Association with the 10 nm Microfibrils
Mark A. Gibson
There is growing evidence that fibrillin-containing microfibrils are not just fibrillin polymers but that a variety of additional macromolecules may be associated with these structures. The functions of these molecules may be envisioned to include a) structural support to stabilize the interactio...
Molecular and Cellular Aspect of Liver Fibrosis
Norifumi Kawada
Hepatic stellate cells play essential roles in the pathogenesis of liver fibrosis. Transformation of stellate cells from the vitamin A-storing pheno type to the “myofibroblastic” one closely correlates to hepatic fibrogenesis during chronic liver diseases. Understanding the molecular mechanisms...
Molecular Developments in the Treatment of Renal Fibrosis
Gavin J. Becker and Tim D. Hewitson
Progressive renal disease is associated with the development of fibrosing lesions not only in the glomerulus, but also in the interstitial and vascular compartments of the kidney, in a process that involves the mesenchymally derived, phenotypically similar, mesangial cell, myofibroblast and vascu...
Mutation Analysis of the FBN1 Gene in Individuals with Marfan Syndrome: Sensitivity, Methods, Clinical Indications
Anne De Paepe, Bart Loeys and Paul Coucke
Since the discovery of the FBN1 gene as the gene responsible for the Marfan syndrome (MFS), molecular testing for this condition has become possible. Although MFS is a clinical diagnosis, certain situations may occur in which molecular analysis of the FBN1 gene is wanted, either for diagnostic...
Myocardial Infarction and Cardiac Fibrosis
Shozo Kusachi and Yoshifumi Ninomiya
Fibrogenesis is essential for infarct healing and affects ventricular remodeling, one of the most important prognostic factors after myocardial infarction. Fibrogenesis is initiated by a variety of cytokines and growth factors produced by activated macrophages and inflammatory cells during the...
Ophthalmological Aspects
Thomas Rosenberg
The eye is involved in a majority of patients affected by Marfan syndrome. Morphological changes essentially affect the microfibrillar elements of the corneoscleral envelope, the iris, and the ciliary zonules, giving rise to clinically and diagnostically significant characteristics. The ophthalmo...
Organization and Biomechanical Properties of Fibrillin Microfibrils
Cay M. Kielty, Tim J. Wess, J. Louise Haston, Michael J. Sherratt, Clair Baldock and C. Adrian Shuttleworth
The evolution and function of multicellular organisms has required tissue flexibility and the necessity to withstand stretch. These properties underpin movement and allow responses to changing environments. The elastic fibre is a bio-composite material composed of two insoluble extracellular matr...
Oxidative Stress, Lipoproteins and Angiotensin II— The Unholy Triade in the Pathogenesis of Renal Fibrosis
Jan Galle, Thomas Quaschning and Stefan Seibold
Renal fibrosis usually indicates irreversible tissue damage, irrespective of the initial cause. Thus, it is most relevant to understand mechanisms leading to renal fibrosis. Oxidative stress has emerged as an important factor contributing to tissue damage, and oxidative stress is enhanced in a va...
Pathological Significance of Renal Expression of Proinflammatory Molecules
Takashi Wada, Mohammed S. Razzaque, Kouji Matsushima, Takashi Taguchi and Hitoshi Yokoyama
Recent studies of cytokines, chemokines, adhesion molecules and growth factors have enhanced our understanding of molecular mechanisms of leukocyte trafficking and their activation in the inflammatory phase of various renal diseases. Interactions between infiltrated inflammatory cells and residen...
Pulmonary Firbogenesis: The Role of Apoptosis and its Clinical Potentials
Kazuyoshi Kuwano, Naoki Hagimata and Nobuyuki Hara
Pulmonary fibrosis is a common response to various insults or injuries to the lung. Al though there are various initiating factors or causes, the terminal stages are characterized by proliferation and progressive accumulation of connective tissue replacing normal functional parenchyma. Conventi...
Recent Therapeutic Developments in Hepatic Fibrosis
Ichiro Shimizu
Currently, hepatic fibrogenesis is viewed as a dynamic process strictly related to the extent and duration of liver injury. Activated hepatic stellate cells (HSCs) are identi fied as the major source of extracellular matrix components in the injured liver, and are regarded as a target for antifib...
Reversibility of Liver Fibrosis: Role of Matrix Metalloproteinases
Isao Okazaki, Tetsu Watanabe, Maki Niioka, Yoshihiko Sugioka and Yutaka Inagaki
Reversibility of liver fibrosis has been reported both experimentally and clinically, if the cause of liver damage is removed or adequately treated. We first reported the collagenase activity in the process of experimental liver fibrosis in 1974. The present review discusses the participation of ...
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