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The Junier blood group system: from discovery to the clinic

hematology
immunology
pathology
pharmacology
medical-genetics

Abstract

Background: In 2012, the International Society of Blood Transfusion (ISBT) formalized the Junier (JR) blood group (ISBT 032), with Jra (ISBT 032001) as its sole antigen. As of May 2025, the ISBT has recognized 48 blood group systems comprising 371 red blood cell antigens encoded by 53 genes. Jra is located on a multipass membrane glycoprotein, adenosine triphosphate (ATP) binding cassette subfamily G member 2 (ABCG2), also known as breast cancer resistance protein (BCRP) or CD338, which is involved in various physiological, biochemical, and metabolic processes in the human body. The glycoprotein is encoded by the ABCG2 gene, located on chromosome 4.

In recent years, polymorphisms in the ABCG2 gene have been increasingly elucidated through deeper investigation of the JR blood group system. Concurrently, the JR blood group system has gained clinical importance due to its association with fetal and neonatal anemia, hydrops fetalis, neonatal jaundice, hemolytic disease of the fetus and newborn (HDFN), hyperuricemia/gout, mirror syndrome, and hemolytic transfusion reaction (HTR).

In addition, the JR blood group system plays a regulatory role in several physiological and pathological processes, including uric acid metabolism, folate and porphyrin homeostasis, substance exchange across the blood-brain barrier, and the transport and resistance of anti-tumor drugs.

This study reviews the distributional characteristics, molecular biological features, immunological features, and clinical significance of the JR blood group system.

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