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Molecular biology of the Rh antigens
P Agre and JP Cartron
Department of Medicine, Johns Hopkins University School of Medicine,
Baltimore, MD 21205.
The RBC Rh antigens are of large clinical importance, but until recently
have been poorly understood at a molecular level. The Rh polypeptides are a
family of nonglycosylated Mr 30- to 32-Kd transmembrane proteins that are
core structural components of the Rh antigens and have been purified and
partially characterized biochemically. Rh polypeptides are present in RBCs
from normal humans and other mammalian species and are probably required
for normal membrane integrity, because they appear to be missing from the
RBCs of the rare Rhnull individuals that express several membrane defects.
The Rh polypeptides contain an exofacial free sulfhydryl that is important
for Rh antigenic reactivity and several intracellular sulfhydryls that
appear to be palmitylated, but most of the molecule appears to reside
between the leaflets of the phospholipid bilayer. The cDNA coding for a
416-amino acid Rh polypeptide was recently isolated but was not found to
share sequence homology with any known protein, and Northern analysis
indicated that Rh is erythroid specific. The Rh antigens within the native
membranes are thought to exist as a complex of Rh polypeptides and multiple
other membrane components, including certain Rh-related glycoproteins.
While it is thought that this assembly may be important for the Rh
antigenic reactivity, the structural basis of this remains to be
established. While the physiologic role of Rh is yet to be defined, several
clues indicate that it may play a role in the organization of membrane
phospholipids or synthesis or membrane expression of various glycoproteins.
While our knowledge of Rh is still very incomplete, recent research has
significantly advanced the molecular understanding of these important blood
group antigens.
Volume 78,
Issue 3,
pp. 551-563,
08/01/1991
Copyright © 1991 by The American Society of Hematology

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