Evidence supporting the requirement for two proline residues for expression of c

Westhoff, Connie M.; Silberstein, Leslie E.; Wylie, Dwane E.

doi:10.1046/j.1537-2995.2000.40030321.x

Cited by 27 publications

(23 citation statements)

References 15 publications

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“…In routine testing, the c antigen was not depressed, which is consistent with previous suggestions that the c epitope(s) structure consisting of two proline residues may be more resistant to conformational changes in the protein. 10 Evidence that RHCE*ceMO also encodes a partial c antigen is demonstrated by the finding of alloanti-c in the plasma of three transfused patients who had RHCE*ceMO in trans to RHCE*Ce. …”

Section: Discussionmentioning

confidence: 99%

RHCEceMO* is frequently in cis to RHDDAU0* and encodes a hr^S–, hr^B–, RH:–61 phenotype in black persons: clinical significance

et al. 2013

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Background RHCE*ceMO has nucleotide changes 48G>C and 667G>T, which encode, respectively, 16Cys and 223Phe associated with altered expression of e antigen. RHD*DAU0 has nt1136C>T, which encodes 379Met associated with normal levels of D. We compiled serologic and DNA testing data on samples with RHCE*ceMO to determine the RBC antigen expression, antibody specificity, RHD association, and the prevalence in African-Americans. Methods Serologic testing was performed by standard methods. Genomic DNA was used for PCR-RFLP and RH- exon sequencing, and for some, Rh-cDNA was sequenced. Seventy-seven (50 donor and 27 patient) samples with RHCE*ceMO were studied, and 350 African-Americans were screened for allele prevalence. Results RBCs from RHCE*ceMO homozygotes (or heterozygotes with RHCE*cE in trans) were weakly or non-reactive with some anti-e , and were non-reactive with polyclonal anti-hrS and anti-hrB. Twenty-three transfused patients homozygous for RHCE*ceMO/ceMO or with RHCE*ceMO in trans to RHCE*cE or *ce had allo anti-e, anti-f, anti-hrS/hrB, or an antibody to a high prevalence Rh antigen. Three patients with allo-anti-c had RHCE*ceMO in trans to RHCE*Ce. RHD*DAU0 was present in 30% of African-Americans tested and in 69 of 77 (90%) of samples with RHCE*ceMO. Conclusions RHCE*ceMO encodes partial e, as previously reported, and also encodes partial c, a hrS– and hrB– phenotype, and the absence of a high prevalence antigen (RH61). The antibody in transfused patients depended on the RHCE allele in trans. RHCE*ceMO was present in 1 in 50 African-Americans with an allele frequency of 0.01, is often linked to RHD*DAU0, and is potentially of clinical significance for transfusion.

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Section: Discussionmentioning

confidence: 99%

RHCEceMO* is frequently in cis to RHDDAU0* and encodes a hr^S–, hr^B–, RH:–61 phenotype in black persons: clinical significance

et al. 2013

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“…Studies with cells of nonhuman primates suggested that of the four amino acids that might be critical for expression of Rhc, P103, the only polymorphic site that is extracellular, plays an important role in expression of c 3,4 . Moreover, we recently described that a single point mutation 307 T>C leading to S103P in RHD resulted in expression of c on the D polypeptide, independent of the presence of W16, L60, and N68 5 .…”

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confidence: 96%

RHC and RHc genotyping in different ethnic groups

et al. 2002

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“…In Caucasians, expression of c antigen is associated with Trp16 (transmembrane), Leu60 (transmembrane), Asn68 (transmembrane), Pro102 (2nd extracellular loop) and Pro103 (2nd extracellular loop) [11,12]. It is well known that the RBC membrane RhD and Rhce proteins are highly conformational and that various extracellular loops interact.…”

Section: Discussionmentioning

confidence: 99%

The low prevalence Rh antigen Be^a(Rh36) is associated withRHCE*ce662C>G in exon 5, which is predicted to encode Rhce 221Arg

et al. 2010

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Background and Objectives The low prevalence antigen, Be a , is produced by a complex that also produces weak c, e and f (ce). We report here the molecular basis associated with Be a antigen expression. Materials and MethodsPeripheral blood samples from four Be(a+) probands were tested. Haemagglutination, gDNA extraction, PCR-based assays, reticulocyte RNA isolation, Rh-cDNA analyses, and sequencing were performed by standard procedures.Results RBCs from Probands 1 and 3 were D)C)E)c+e+, and from Probands 2 and 4 were D+C+E)c+ W e+. In proband 1, cDNA sequencing of RHCE revealed heterozygosity of nucleotide (nt) 662C ⁄ G in exon 5 of RHCE*ce. No other nucleotide changes were observed. As the 662C>G nucleotide change ablates a MscI restriction enzyme cleavage site, PCR-RFLP analysis was performed and the RHCE*ce nt 662C ⁄ G heterozygosity was detected on gDNA from the four probands and two children from both Proband 3 and Proband 4. ConclusionThe low prevalence Rh antigen, Be a , is associated with a single nucleotide change in exon 5 of RHCE*ce; that of 662C>G and this change is predicted to alter proline at amino acid position 221 of Rhce to arginine. The fundamental differences in the properties of these two amino acids may impose a steric and ⁄ or charge-related effect on the protein, and thereby provide an explanation for the weakened expression of c, e and f (ce) antigens in the Be a phenotype.

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Evidence supporting the requirement for two proline residues for expression of c

Cited by 27 publications

References 15 publications

RHCEceMO* is frequently in cis to RHDDAU0* and encodes a hr^S–, hr^B–, RH:–61 phenotype in black persons: clinical significance

RHCEceMO* is frequently in cis to RHDDAU0* and encodes a hr^S–, hr^B–, RH:–61 phenotype in black persons: clinical significance

RHC and RHc genotyping in different ethnic groups

The low prevalence Rh antigen Be^a(Rh36) is associated withRHCE*ce662C>G in exon 5, which is predicted to encode Rhce 221Arg

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Evidence supporting the requirement for two proline residues for expression of c

Cited by 27 publications

References 15 publications

RHCE*ceMO is frequently in cis to RHD*DAU0 and encodes a hrS–, hrB–, RH:–61 phenotype in black persons: clinical significance

RHCE*ceMO is frequently in cis to RHD*DAU0 and encodes a hrS–, hrB–, RH:–61 phenotype in black persons: clinical significance

RHC and RHc genotyping in different ethnic groups

The low prevalence Rh antigen Bea(Rh36) is associated withRHCE*ce662C>G in exon 5, which is predicted to encode Rhce 221Arg

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RHCEceMO* is frequently in cis to RHDDAU0* and encodes a hr^S–, hr^B–, RH:–61 phenotype in black persons: clinical significance

RHCEceMO* is frequently in cis to RHDDAU0* and encodes a hr^S–, hr^B–, RH:–61 phenotype in black persons: clinical significance

The low prevalence Rh antigen Be^a(Rh36) is associated withRHCE*ce662C>G in exon 5, which is predicted to encode Rhce 221Arg