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Molecular analysis of Rh transcripts and polypeptides from individuals
expressing the DVI variant phenotype: an RHD gene deletion event does not
generate All DVIccEe phenotypes
ND Avent, W Liu, JW Jones, ML Scott, D Voak, M Pisacka, J Watt and A Fletcher
International Blood Group Reference Laboratory, Southmead, Bristol, UK.
The D antigen is a mosaic comprising at least 30 epitopes. Partial Rh D
phenotypes occur when there is absence of one or more of these epitopes,
with the remainder expressed. The DVI phenotype is the most common of the
partial D phenotypes, lacking most D antigen epitopes (ep D) (epD1, 2, 5-8
using the 9-epitope model or epD 1-4, 7-22, 26-29 using the 30-epitope
model). DVI mothers may become immunized by transfusion with D-positive
blood (if typed as D-positive using polyclonal typing reagents) or by
fetuses which have all of the D antigen. This situation can give rise to
severe hemolytic disease of the newborn (HDN). The molecular basis of the
DVI phenotype has previously been proposed to occur by two different
genetic mechanisms, one (in individuals of DVICcee phenotype) where a gene
conversion event generates a hybrid RHD-RHCE-RHD gene; the second (in
individuals of DVIccEe phenotype) was proposed to be caused by a partial
RHD gene deletion. We present evidence that in four DVICcee phenotypes
studied, this phenotype is not generated by a partial RHD gene deletion,
but occurs by a similar mechanism to the DVICcee phenotypes. In two
individuals we have found hybrid RHD-RHCE-RHD transcripts in both DVICe and
DVIcE haplotypes. These differ in that the DVICe transcripts are derived
from an RHD gene where exons 4-6 have been replaced with RHCE equivalents
(encoding Ala226); the DVIcE transcripts are derived from an RHD gene where
exons 4 and 5 are replaced by RHCE equivalents (encoding Pro226). We
provide direct evidence that Rh DVI polypeptides are expressed at the
erythrocyte surface as full-length polypeptide products. We have used
immunoprecipitation experiments using anti-D reactive with DVI erythrocytes
followed by immunoblotting the immune complexes with rabbit sera
immunoreactive to the fourth external and C- terminal domains of all Rh
polypeptides. Our results illustrate that these domains are present on all
Rh DVI proteins studied, and suggest that Rh DVI polypeptide species
studied here exist as full-length Rh proteins.
Volume 89,
Issue 5,
pp. 1779-1786,
03/01/1997
Copyright © 1997 by The American Society of Hematology
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