PNH and the GPI-Linked Proteins
Neal S. Young, Joel Moss
Academic Press, 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 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
abnormal Acad acid acidified serum activity acyl alternative pathway amino antibody antigen aplastic anemia binding Biochem biosynthesis Blood bone marrow brucei C3 convertase caveolae cDNA Cell Biol cell lines cell surface Chem cholesterol Clin clinical clone complement complex component cross-linking Dacie deficiency domains enzyme erythrocytes Exon expression factor Frameshift function glycan glycoprotein glycosyl glycosylphosphatidylinositol GPI-anchored proteins GPI-PLD GPIAPs Haematol hematopoietic hemolysis hemolytic human immune Immunol inhibition inhibitor inositol integrin intracellular Kinoshita lipid lymphocytes lysis mammalian mediated Medof membrane anchor membrane proteins mice MIRL Missense molecular molecules Nafa Natl normal parasite paroxysmal nocturnal hemoglobinuria patients with PNH phenotype phosphatidylinositol phospholipase PI-PLC PIG-A gene PIG-A mutations PIGA plasma membrane platelets PNH erythrocytes PNH patients Pramoonjago Proc properdin rafts reactive lysis receptor red cells residues role Rosse RT-PCR sensitivity sequence signal transduction somatic SSCA stem cells structure studies Takeda thrombosis transmembrane tyrosine kinases uPAR vitro vivo