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Biochemical studies on red blood cells from a patient with the Inab
phenotype (decay-accelerating factor deficiency)
ME Reid, G Mallinson, RB Sim, J Poole, V Pausch, AH Merry, YW Liew and MJ Tanner
International Blood Group Reference Laboratory, Bristol, UK.
A 38-year-old Russian woman (KZ) has been identified as the fourth
proposita with the Inab blood group phenotype. Like the first two
propositi, she has a chronic intestinal disorder and, as shown for the
third proposita, her Inab phenotype is demonstrably inherited. KZ's serum
contained anti-IFC, which reacted with a red blood cell (RBC) membrane
component with an Mr of 70,000, which is decay accelerating factor (DAF).
Her RBCs lacked all Cromer-related blood group antigens and DAF. Her RBCs
were no more susceptible than normal control RBCs to lysis in acid lysis or
in rabbit or human antibody-initiated complement lysis tests. Northern
blots of total RNA isolated from KZ's Epstein- Barr virus-transformed
lymphoblasts showed a marked reduction of DAF mRNA when compared with
normal. Polymerase chain reaction (PCR) amplification of cDNA confirmed
this reduced level of DAF mRNA. Sequencing of the PCR product showed a
44-nucleotide deletion in the mRNA close to the short consensus repeats
IIIa/IIIb intron/exon boundary. This deletion results in a change in the
reading frame that places a termination codon six amino acids after the
deletion. The putative translation product would lack a glycosyl
phosphatidyl- inositol linkage site and, therefore, would not be
membrane-bound in the RBC.
Volume 78,
Issue 12,
pp. 3291-3297,
12/15/1991
Copyright © 1991 by The American Society of Hematology
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