基本説明
Describes the clinical features of PNH: Hemolysis, thrombosis, and aplastic anemia, and explains the unusual mechanism of erythrocyte destruction.
Full Description
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. It outlines the chemical features of PNH; explains the mechanism of hemolysis; includes work on the biochemistry of glycophosphoinositol anchors; and contains descriptions of the chemistry and biophysics of GPI-anchored proteins.
