An update on the Scianna blood group system

Brunker, Patricia A. R.; Flegel, Willy A.

doi:10.21307/immunohematology-2020-012

Cited by 5 publications

(5 citation statements)

References 18 publications

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“…The Scianna (SC) blood group system is the 13th recognized by the International Society of Blood Transfusion (ISBT). It consists of seven high prevalence antigens, Sc1 (formerly Sm), Sc3, STAR (SC5), SCER (SC6), SCAN (SC7), SCAR (SC8), 1 and SCAC (SC9) (recognized by ISBT and detailed in the present report) and two of low prevalence, Sc2 (formerly Bu a ) and Rd (SC4) 2,3 . The most prevalent phenotype is SC: 1, –2, with SC: –1, 2 and SC: 1, 2 being very rare.…”

Section: Introductionmentioning

confidence: 75%

“…Most people with SC: –1, –2, –3 phenotypes have originated from the Marshall Islands or other Pacific Islands and the antibody they make is anti‐Sc3. Antibodies in the Scianna system may cause clinically significant hemolysis, albeit rarely 2,3 …”

Section: Introductionmentioning

confidence: 99%

“…It consists of seven high prevalence antigens, Sc1 (formerly Sm), Sc3, STAR (SC5), SCER (SC6), SCAN (SC7), SCAR (SC8), 1 and SCAC (SC9) (recognized by ISBT and detailed in the present report) and two of low prevalence, Sc2 (formerly Bu a ) and Rd (SC4). 2,3 The most prevalent phenotype is SC: 1, -2, with SC: -1, 2 and SC: 1, 2 being very rare. Two SC null alleles (designated with SC*01N) and associated with a SC: -1, -2, -3 phenotype due to absence of the protein are currently listed by the ISBT.…”

Section: Introductionmentioning

confidence: 99%

“…Antibodies in the Scianna system may cause clinically significant hemolysis, albeit rarely. 2,3 Scianna antigens are expressed by the erythrocyte membrane-associated protein (ERMAP) 4 a member of the immunoglobulin superfamily based on structural similarity, and more specifically of the Butyrophilins/ Myelin Oligodendrocyte Glycoprotein (or BTN/MOG) family. 6 The precise role of ERMAP in human RBCs is unresolved.…”

Section: Introductionmentioning

confidence: 99%

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Two new Scianna variants causing loss of high prevalence antigens: ERMAP model and 3D analysis of the antigens

et al. 2022

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Background Scianna (Sc) antigens, seven high and two of low prevalence, are expressed on erythrocyte membrane‐associated protein (ERMAP). We investigated SC (ERMAP) in individuals who made antibodies to high prevalence Scianna antigens, and propose a 3D model for ERMAP to precisely localize the residues associated with the known antigens. Methods Serological testing and DNA sequencing was performed by standard methods. A 3D structural model was built using a multi‐template homology approach. Protein structures representing missense variants associated with the loss or gain of an antigen were generated. Residue accessibility and intraprotein interactions were compared with the wild‐type protein. Results Two new SC alleles, one with c.349C > T (p.Arg117Cys) in a woman from South India with anti‐Sc3 in her plasma, and a c.217_219delinsTGT (p.Arg73Cys) in an African‐American woman with an antibody to a new high prevalence antigen, termed SCAC, were identified. Six structural templates were used to model ERMAP. 3D analysis showed that residues key for Scianna antigen expression were all exposed at the surface of the extracellular domain. The p.Arg117Cys change was predicted to abolish interactions between residues 93 and 117, with no compensating interactions. Conclusion We confirm the extracellular location of Scianna residues responsible for antigen expression which predicts direct accessibility to antibodies. Loss of intraprotein interactions appear to be responsible for a Sc null and production of anti‐Sc3 with p.117Cys, SC*01 N.03, and for loss of a high prevalence antigen with p.73Cys, termed SCAC for Sc Arg to Cys. Comparative modeling aids our understanding of new alleles and Scianna antigen expression.

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Section: Introductionmentioning

confidence: 75%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Two new Scianna variants causing loss of high prevalence antigens: ERMAP model and 3D analysis of the antigens

et al. 2022

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“…11 It is known as the "lucky thirteenth" among blood group systems. 12 https://doi.org/10.29309/TPMJ/2023.30.03.7321…”

Section: Introductionmentioning

confidence: 99%

Genotyping for Scianna blood group system polymorphism in Pakistan.

Jadoon

Muhammad

Aslam

et al. 2023

TPMJ

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Objective: To establish the occurrence of Scianna blood group alleles via genotyping in Pakistani blood donors. Study Design: Cross-sectional Analytical study. Setting: Department of Hematology, Army Medical College (AMC) in association with the Armed Forces Institute of Transfusion (AFIT) Rawalpindi. Period: January 2019 and January 2020. Material & Methods: In this study 300 blood donors were incorporated after taking informed consent; DNA was extracted from their EDTA anticoagulated blood samples and tested for SC*01 and SC*02 alleles by Sequence-Specific Primer Polymerase Chain Reaction (PCR-SSP), which was ensued by gel electrophoresis. The allele frequencies were established and Hardy Weinberg equilibrium was determined. Chi-square test was applied to compare the observed and expected genotype frequencies as well as for comparison with published allele frequencies of other populations. We used SPSS version 22 for the analysis of study data. Results: All donors were homozygous for SC*01, demonstrating allele frequency for SC*01 nearly 1.00. Genotype frequencies were congruent with the Hardy Weinberg equilibrium. The difference between the allele frequencies of SC*01 and SC*02 alleles of population of Pakistan was not statistically significant when compared to those of other populations. Conclusion: This maiden research illustrates Scianna blood group distribution in Pakistani community by PCR-SSP. This method is cheap and proficient and can be applied in developing nations. The findings of this study can aid in the production of local red cell panels, detection of uncommon blood types, and setting up of a domestic rare blood donor platform.

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SCAR: The high‐prevalence antigen 013.008 in the Scianna blood group system

et al. 2020

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Background: The Scianna (SC) blood group system comprises seven antigens. They reside on the erythroblast membrane-associated glycoprotein (ERMAP). The ERMAP and RHCE genes are juxtaposed to each other on chromosome 1. We report a novel SC antigen. Study Design and Methods: Blood samples came from a patient and his two sisters in Saudi Arabia. To investigate the antibody specificity we used the column agglutination technique and soluble recombinant ERMAP protein. The significance of anti-SCAR was evaluated by the transfusion history and a monocyte monolayer assay. We determined the genomic sequence of ERMAP and RHCE genes. Results: The patientʼs serum showed an antibody of titer 8 against a highprevalence antigen. The soluble recombinant ERMAP protein inhibited the antibody. The propositus genotyped homozygous for an ERMAP:c.424C>G variant, for which his sisters were heterozygous. The c.424C>G variant occurred in the SC*01 allele in one haplotype with the RHCE*03 (RHCE*cE) allele. No signs of hemolysis occurred following an incompatible blood transfusion. The monocyte monolayer assay was negative. Conclusions: We characterized a high-prevalence antigen, with the proposed name "SCAR," which is the eighth antigen of the Scianna blood group system (proposed designation 013.008). Individuals homozygous for ERMAP:p. (Gln142Glu) protein variant can produce anti-SCAR. Although we did not observe any sign of hemolysis at this time, the anti-SCAR prompted a change of the treatment regimen. A review of the known reports indicated that all SC alloantibodies of sufficient titer should be considered capable of causing hemolysis.

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An update on the Scianna blood group system

Cited by 5 publications

References 18 publications

Two new Scianna variants causing loss of high prevalence antigens: ERMAP model and 3D analysis of the antigens

Two new Scianna variants causing loss of high prevalence antigens: ERMAP model and 3D analysis of the antigens

Genotyping for Scianna blood group system polymorphism in Pakistan.

SCAR: The high‐prevalence antigen 013.008 in the Scianna blood group system

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