Cover of: PNH and the GPI | Read Online

PNH and the GPI Linked Proteins by

  • 12 Want to read
  • ·
  • 52 Currently reading

Published by Academic Press .
Written in English


  • Cellular biology,
  • Genetics (non-medical),
  • Haematology,
  • Medical / Nursing,
  • Medical,
  • Biochemistry,
  • Hematology,
  • Oncology,
  • Medical / Hematology,
  • Cellular signal transduction,
  • Glycoproteins,
  • Membrane proteins,
  • Paroxysmal hemoglobinuria,
  • Phosphoinositides

Book details:

Edition Notes

ContributionsNeal S. Young (Editor), Joel Moss (Editor)
The Physical Object
Number of Pages296
ID Numbers
Open LibraryOL9866864M
ISBN 100127729402
ISBN 109780127729404

Download PNH and the GPI


  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 .