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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
medicine
Partial Ankyrin and Spectrin Deficiency in Severe, Atypical Hereditary Spherocytosis
New England Journal of Medicine, Volume 318, No. 4, Year 1988
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Description
HEREDITARY spherocytosis is a common form of hemolytic anemia that is heterogeneous in terms of its clinical presentation, molecular basis, and inheritance.1 The primary defect is thought to reside in the red-cell membrane skeleton, a submembranous network composed mainly of spectrin, actin, and proteins that migrate on gel electrophoresis as bands 4.1 and 4.9 (proteins 4.1 and 4.9).2 Visualization of the skeleton by electron microscopy has revealed a primarily hexagonal lattice of fibers of spectrin tetramers linked to junctional complexes containing actin and proteins 4.1 and 4.9.3 4 5 The skeleton is attached to the membrane by ankyrin (protein 2.1), which connects. © 1988, Massachusetts Medical Society. All rights reserved.
Authors & Co-Authors
Coetzer, Thérèsa Louise
United States, Boston
Tufts University School of Medicine
United States, Birmingham
The University of Alabama at Birmingham
Canada, Hamilton
Mcmaster University
United Kingdom, London
Imperial College Faculty of Medicine
Prchal, Josef T.
United States, Boston
Tufts University School of Medicine
United States, Birmingham
The University of Alabama at Birmingham
Canada, Hamilton
Mcmaster University
United Kingdom, London
Imperial College Faculty of Medicine
Dacie, John V.
United States, Boston
Tufts University School of Medicine
United States, Birmingham
The University of Alabama at Birmingham
Canada, Hamilton
Mcmaster University
United Kingdom, London
Imperial College Faculty of Medicine
Palek, Jiří
United States, Boston
Tufts University School of Medicine
United States, Birmingham
The University of Alabama at Birmingham
Canada, Hamilton
Mcmaster University
United Kingdom, London
Imperial College Faculty of Medicine
Statistics
Citations: 71
Authors: 4
Affiliations: 4
Identifiers
Doi:
10.1056/NEJM198801283180407
ISSN:
00284793