PNH and the GPI-Linked Proteins

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Neal S. Young, Joel Moss
Elsevier, Apr 28, 2000 - Medical - 279 pages
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.
  • 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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Chapter 1 A Brief History of PNH
Chapter 2 Genetics of PNH
Chapter 3 Hemolysis in PNH
Chapter 4 Thrombotic Complications in PNH
Chapter 5 Bone Marrow Failure in PNH
Chapter 6 Animal Models of PNH
Chapter 7 The Function of GPIAnchored Proteins
Chapter 8 GPI in Lower Animals
Chapter 9 Synthesis of the GPI Anchor
Chapter 10 Functional and Structural Organization of GPIAnchored Proteins in Cellular Membranes
Chapter 11 Structure and Function of GPISpecific Phospholipases
Sequence of the Coding Region of the Human PIGA Gene

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Page 17 - Ham, TH, and Dingle, JH: Studies on destruction of red blood cells. II. Chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria: Certain immunological aspects of the hemolytic mechanism with special reference to serum complement.

About the author (2000)

Neal S. Young is Chief of the Hematology Branch of the National Heart, Lung, and Blood Institute at the National Institutes of Health, Bethesda, Maryland.

National Institutes of Health, Bethesda, MD, USA

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