Molecular and cellular aspects of the serpinopathies and disorders in serpin activity / editors, Gary A. Silverman, David A. Lomas.

Format:

Book

Contributor:

Silverman, Gary A.

Lomas, David A.

Language:

English

Subjects (All):

Serpins.

Serpins--Physiological effect.

Physical Description:

xxix, 639 pages : illustrations (some color) ; 24 cm

Place of Publication:

Singapore : World Scientific Pub., [2007]

Summary:

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.

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, James C. Whisstock 1

Chapter 2 Serpin Conformations / Mary C. Pearce, Robert N. Pike, Arthur M. Lesk, Stephen P. Bottomley 35

Chapter 3 Mechanisms of Serpin Inhibition / Peter G. W. Gettins 67

Section II Lessons from Model Organisms

Chapter 4 Mouse Serpins and Transgenic Studies / David J. Askew, Paul Coughlin, Phillip I. Bird 101

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, James C. Whisstock 131

Chapter 6 New Lessons from Poxvirus Serpins / Peter C. Turner, Priscilla F. McAuliffe, Amy L. MacNeill, Richard W. Moyer 163

Chapter 7 The Intracellular Serpins of Caenorhabditis elegans / Stephen C. Pak, Yuko S. Askew, Cliff J. Luke 195

Chapter 8 Drosophila Serpins: Regulatory Cascades in Innate Immunity and Morphogenesis / David Gubb, Andrew Robertson, Tim Dafforn, Laurent Troxler, Jean-Marc Reichhart 207

Chapter 9 Serpins in a Lepidopteran Insect, Manduca sexta / Michael R. Kanost 229

Chapter 10 MENT, a Chromatin-Associated Serpin from Avian Blood Cells: Structure and Function / Sergei A. Grigoryev, Yaroslava A. Bulynko, Robert N. Pike, James C. Whisstock 243

Chapter 11 Uterine Serpins / Peter J. Hansen, Saban Tekin, Maria B. Padua 261

Chapter 12 Plant Serpins / Jorn Hejgaard, Thomas H. Roberts 279

Section III Serpin Physiology and Pathophysiology

Chapter 13 Serpins, Apoptosis and Other Aspects of Cell Death / Fiona L. Scott 301

Chapter 14 Serpins in Malignancy: Tumor Cell Invasion, Motility and Angiogenesis / Philip A. Pemberton, Christian Schem, Nicolai Maass, Ming Zhang 337

Chapter 15 Serpins in Malignancy: Prognostic Indicators / Hiroshi Kato, Yuko S. Askew, Shugo Nawata, Akihiro Murakami, Yoshinori Suminami, Gary Clayman 363

Chapter 16 SERPINB7 (Megsin) Nephropathy / Toshio Miyata 383

Chapter 17 Serpin-Protein and Receptor Interactions / Toni M. Antalis, Dudley K. Strickland 393

Chapter 18 Serpins and the Metabolic Syndrome / Jun Wada 411

Chapter 19 Serpin Interactions with Bacterial Peptidases / Jan Potempa, Tomasz Kantyka, Daniel Nelson 425

Section IV Congenital Deficiencies and Serpinopathies

Chapter 20 The Serpinopathies and Respiratory Disease / David A. Lomas, Didier Belorgey, Elena Miranda, Meera Mallya, Peter Hagglof, Lynda K. Sharp, Russell L. Phillips, Richard Page, Mark J. Davies, Damian C. Crowther 445

Chapter 21 Liver Disease in [alpha subscript 1]-Antitrypsin Deficiency / David H. Perlmutter 483

Chapter 22 Regulation of Hemostasis by Heparin-Binding Serpins / Frank C. Church, Robert N. Pike, Douglas M. Tollefsen, Ashley M. Buckle, Angelina V. Ciaccia, Steven T. Olson 509

Chapter 23 C1 Inhibitor: Structure, Function, Biologic Activity and Angioedema / Philip A. Patston, Alvin E. Davis III 555

Chapter 24 Neuroserpin in Neurological Disease / Manuel Yepes, Daniel A. Lawrence 593

Chapter 25 Serpins and Alzheimer's Disease / H. Tonie Wright, Sabina Janciauskiene 619.

Notes:

Includes bibliographical references and index.

ISBN:

9812569634

9789812569639

OCLC:

145553766