Genes of the Rh System

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The page below is a sample from the LabCE course Overview Of Major Antigens of the Rh Blood Group System (retired 2/12/2020). Access the complete course and earn ASCLS P.A.C.E.-approved continuing education credits by subscribing online.

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Genes of the Rh System

Due to its complexities, the Rh system continues to be studied extensively. As predicted, RH genes are inherited as codominant alleles. The current explanation of genetic control of the system is that there are two closely linked genes for the Rh system located on chromosome 1. The RHD gene encodes for the RhD protein while the RHCE gene encodes for the following Rh proteins - RhCe, Rhce, RhcE, or RhCE. Alterations in the amino acid sequences of the RHD gene result in a high degree of variability in the expression of the D antigen on human RBCs.
RHAG is a gene that resides on chromosome 6 and results in a protein referred to as the RH-associated glycoprotein (RhAG). Within the red cell membrane RhAG forms complexes with other Rh proteins. Alone, the glycoprotein does not result in any antigen expression, but must be present for expression of the Rh antigens. Mutations in this gene may result in missing or altered Rh proteins.

Blaney, KD and Howard, PR: Basic & Applied Concepts of Blood Banking and Transfusion Practices, 3rd ed. Elsevier, St. Louis, MO, 2013, p 108.
Harmening, DM,: Modern Blood Banking & Transfusion Practices, 6th ed. FA Davis, Philadelphia, PA, 2012, p 157.