Published February 2000
by Academic Press .
Written in English
|Contributions||Neal S. Young (Editor), Joel Moss (Editor)|
|The Physical Object|
|Number of Pages||296|
Purchase PNH and the GPI-Linked Proteins - 1st Edition. Print Book & E-Book. ISBN , Book Edition: 1. 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 . PNH arises from acquired mutations in the PIG-A gene. Affected individuals have hematopoietic stem cells (HPSC) that are deficient in glycosylphosphatidylinositol (GPI)-anchored proteins (GPI-AP), such Format: Kindle. PAROXYSMAL NOCTURNAL hemoglobinuria (PNH) is an acquired, clonal somatic stem cell disorder associated with hemolysis, thrombosis, and bone marrow failure.1 PNH is caused by somatic cell mutations in a gene called PIG-A that produces global deficiency of a class of proteins linked to the cell surface by a glycosylphosphatidylinositol (GPI) anchor.2,3 A large and diverse group of GPI .
Characteristics of PNH •PNH is not a binary process •The clinical manifestations are determined primarily by: –The size of the PNH clone •The peripheral blood of patients is a mosaic of normal and abnormal cells –The degree of deficiency of GPI-APs •Some cells are completely deficient in GPI . Paroxysmal nocturnal hemoglobinuria (PNH) is a rare but often debilitating disease which may lead to death in up to 35% of patients within 5 years if unrecognized and untreated. Part of the Methods in Molecular Biology book series (MIMB, volume Sutherland DR et al () ICCS/ESCCA consensus guidelines to detect GPI . PNH is the subject of a great deal of current research, highly illustrative of the mutually productive intersection of clinical studies and basic science, PNH and the GPI-Linked Proteins is the first book Format: Hardcover. PNH is a rare disease, but normal individuals harbor tiny PNH clones: very small numbers, /million, of GPI-anchored proteindeficient, PIG-A-granulocytes were detected after re-peated cell.
A partial list GPI-linked proteins includes CD14, CD16, CD24, CD55, CD56, CD58, CD59, C8-binding protein, alkaline phosphatase, acetylcholine esterase, and a variety of high frequency human blood group antigens. In PNH, erythrocytes, platelets, granulocytes, and monocytes have a partial or total lack of these GPI . Paroxysmal Nocturnal Hemoglobinuria (PNH) is understood at the level of the gene and the protein. 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 . This book, Paroxysmal Nocturnal Hemoglobinuria, discusses the direction of continuing research in this area, as well as the potential for the development of management guidelines. It serves as a valuable source of information for both basic scientists and physicians, especially immunologists targeting GPI . Parker CJ. Historical Aspects of Paroxysmal Nocturnal Hemoglobinuria: “Defining the Disease.” Brit J Haematol , What causes PNH? What causes PNH? PNH requires “two-hits” 1) A mutation must occur in a hematopoietic stem cell. Partial or complete deficiency of the GPI anchor 2) PNH .