PNH and the GPI-Linked Proteins

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Neal S. Young, Joel Moss
Academic Press, Apr 28, 2000 - Medical - 279 pages
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Paroxysmal Nocturnal Hemoglobinuria (PNH) has been recognized for over a century. This mysterious disease is now understood at the level of the gene and the protein. The pathophysiology is related to a class of cell surface proteins with distinctive biochemical and physical characteristics. Recently it has been acknowledged that PNH is not rare, and once sensitive assays--based on the chemistry of the proteins--can be applied to many patients.
Written by international experts in the field, this book includes a number of distinctive characteristics, such as the clinical features of PNH, the mechanism of hemolysis, the biochemistry of glycosylphosphoinositol anchors, and the chemistry and biophysics of GPI-anchored proteins.
This unique and timely volume will have a wide audience, including hematologists and oncologists with a clinical interest in this disease, as well as basic biochemists, immunologists, and cell biologists studying this class of proteins.

Key Features
* Outlines the chemical features of PNH
* Explains the mechanism of hemolysis
* Includes work on the biochemistry of glycophosphoinositol anchors
* Contains descriptions of the chemistry and biophysics of GPI-anchored proteins

  

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Contents

Chapter 1 A Brief History of PNH
1
Chapter 2 Genetics of PNH
21
Chapter 3 Hemolysis in PNH
49
Chapter 4 Thrombotic Complications in PNH
101
Chapter 5 Bone Marrow Failure in PNH
113
Chapter 6 Animal Models of PNH
139
Chapter 7 The Function of GPIAnchored Proteins
159
Chapter 8 GPI in Lower Animals
179
Chapter 9 Synthesis of the GPI Anchor
199
Chapter 10 Functional and Structural Organization of GPIAnchored Proteins in Cellular Membranes
221
Chapter 11 Structure and Function of GPISpecific Phospholipases
239
Sequence of the Coding Region of the Human PIGA Gene
269
Index
271
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