PNH and the GPI-Linked Proteins

ISBN 13: 9780123908018

PNH and the GPI-Linked Proteins

 
9780123908018: PNH and the GPI-Linked Proteins

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.

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From the Back Cover:

Paroxysmal nocturnal hemoglobinuria (PNH) was first recognized by a clinician in the mid-19th century. A mysterious syndrome that can combine in a single patient severe red blood cell destruction, life-threatening thrombosis, and marrow failure, PNH is now intimately understood at the level of the gene and proteins. The pathophysiology is related to absence of an entire class of cell surface proteins with highly distinctive biochemical and physical characteristics. All PNH patients show mutations or deletions within a single gene. PPIG-A, arising n a hemotopoietic stem cell. With sensitive assays based on the distinctive biochemistry of the disease, PNH clones can now be recognized in much larger numbers of patients, and small clones appear to exist in most normal individuals as well. These discoveries have enormous importance for the mechanism of disease and for fundamental cell biology.

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 devoted to these important new findings.

Written by international experts in the field, this unique and timely volume will appeal to hematologists and oncologists with a clinical interest in this disease, as well as to basic biochemists, immunologists, and cell biologists studying this class of proteins.

Key Features

  • Describes the clinical features of PNH: hemolysis, thrombosis, and aplastic anemia
  • Explains the unusual mechanism of erythrocyte destruction
  • Includes descriptions of the biochemistry of glycosylphosphoinoisitol-anchored proteins and of the anchor structure

About the Author:

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.

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Published by Academic Press
ISBN 10: 0123908019 ISBN 13: 9780123908018
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