Molecular and Cellular Aspects of the Serpinopathies and Disorders in Serpin Activity 1st Edition by Gary Silverman, Professor Respiratory Medicine Unit David A Lomas ISBN 978-9812569639 9812569634
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Product details:
ISBN 10: 9812569634
ISBN 13: 978-9812569639
Author: Gary A. Silverman, Professor Respiratory Medicine Unit David A Lomas
Serpins constitute a superfamily of proteins that possess a unique tertiary structure and mechanism of proteinase inhibition. In humans, serpins constitute 10% of the plasma proteins and are best known as critical regulators of both the thrombotic and fibrinolytic systems. Serpins also participate in the regulation of the complement cascade, angiogenesis, tumor metastasis, apoptosis and innate immunity. Considering the importance of these molecules in regulating proteolytic cascades, it is not surprising to find that loss- and gain-of-function mutations result in significant human diseases.Massive thrombosis or bleeding, hereditary angioedema, Alzheimer’s disease, diabetic angiopathy and tumor invasion are some of the human diseases associated with serpins. In addition, mutations that alter serpin conformations (the serpinopathies) lead to lung disease, cirrhosis and a form of familial dementia. The goal of this text is to present the current knowledge on the molecular and cellular basis of serpins and their diseases.
Table of contents:
SECTION I
OVERVIEW OF THE SERPIN SUPERFAMILY
Chapter 1
Evolution and Classification of the
Serpin Superfamily
James A. Irving, Lisa D. Cabrita, Dion Kaiserman, Margaret M. Worrall and James C. Whisstock
Chapter 2
Serpin Conformations
Mary C. Pearce, Robert N. Pike, Arthur M. Lesk and Stephen P. Bottomley
Chapter 3
Mechanisms of Serpin Inhibition
Peter G. W. Gettins
SECTION II
LESSONS FROM MODEL ORGANISMS
Chapter 4
Mouse Serpins and Transgenic Studies
David J. Askew, Paul Coughlin and Phillip I. Bird
Chapter 5
Serpins in Prokaryotes
Qingwei Zhang, Ruby Law, Ashley M. Buckle, Lisa Cabrita, Sheena McGowan, James A. Irving. Noel G. Faux, Arthur M. Lesk, Stephen P. Bottomley and James C. Whisstock
Chapter 6
New Lessons from Poxvirus Serpins
Peter C. Turner, Priscilla F. McAuliffe, Amy L. MacNeill, and Richard W. Moyer
Chapter 7
The Intracellular Serpins of Caenorhabditis elegans
Stephen C. Pak, Yuko. S. Askew and Cliff J. Luke
Chapter 8
Drosophila Serpins: Regulatory Cascades in Innate Immunity and Morphogenesis
David Gubb, Andrew Robertson, Tim Dafforn, Laurent Troxler and Jean-Marc Reichhart
Chapter 9
Serpins in a Lepidopteran Insect, Manduca sexta
Michael R. Kanost
Chapter 10
MENT, a Chromatin-Associated Serpin from Avian Blood Cells: Structure and Function
Sergei A. Grigoryev, Yaroslava A. Bulynko, Robert N. Pike and James C. Whisstock
Chapter 11
Uterine Sprinkles
Peter J. Hansen, Saban Tekin and Maria B. Padua
Chapter 12
Plant Serpins
Jørn Hejgaard and Thomas H. Roberts
SECTION III
SERPIN PHYSIOLOGY AND PATHOPHYSIOLOGY
Chapter 13
Serpins, Apoptosis and Other Aspects of Cell Death
Fiona L. Scott
Chapter 14
Serpins in Malignancy: Tumor Cell Invasion, Motility and Angiogenesis
Philip A. Pemberton, Christian Schem, Nicolai Maass and Ming Zhang
Chapter 15
Serpins in Malignancy: Prognostic Indicators
Hiroshi Kato, Yuko S. Askew, Shugo Nawata, Akihiro Murakami, Yoshinori Suminami and Gary Clayman
Chapter 16
SERPINB7 (Megsin) Nephropathy
Toshio Miyata
Chapter 17
Serpin-Protein and Receptor Interactions
Toni M. Antalis and Dudley K. Strickland
Chapter 18
Serpins and the Metabolic Syndrome
Jun Wada
Chapter 19
Serpin Interactions with Bacterial Peptidases
Jan Potempa, Tomasz Kantyka and Daniel Nelson
SECTION IV
CONGENITAL DEFICIENCIES AND SERPINOPATHIES
Chapter 20
The Serpinopathies and Respiratory Disease
David A. Lomas, Didier Belorgey, Elena Miranda, Meera Mallya, Peter Hägglöf, Lynda K. Sharp, Russell L. Phillips, Richard Page, Mark J. Davies and Damian C. Crowther
Chapter 21
Liver Disease in a₁-Antitrypsin Deficiency
David H. Perlmutter
Chapter 22
Chapter 23
Regulation of Hemostasis by Heparin-Binding Serpins
Frank C. Church, Robert N. Pike, Douglas M. Tollefsen, Ashley M. Buckle, Angelina V. Ciaccia and Steven T. Olson
C1 Inhibitor: Structure, Function, Biologic
Activity and Angioedema
Philip A. Patston and Alvin E. Davis III
Chapter 24
Neuroserpin in Neurological Disease
Manuel Yepes and Daniel A. Lawrence
Chapter 25
Serpins and Alzheimer’s Disease
H. Tony Wright and Sabina Janciauskiene
Index
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Tags: Gary Silverman, Professor Respiratory Medicine Unit David A Lomas, Molecular and Cellular, the Serpinopathies, Serpin Activity