rs36020563

This variant is located in the HBB gene.

ClinVar annotation

Pathogenic
1 submitter6 publications

Erythrocytosis, familial, 6

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Research that mentions this SNP (1)

Abnormal hemoglobins with high oxygen affinity and erythrocytosis
ReviewWajcman H. et al.(1996)· Hematology and Cell Therapy

This review comprehensively surveys over 200 hemoglobin variants with high oxygen affinity reported since 1966. Approximately one-third of these variants cause compensatory erythrocytosis (polycythemia), with severity depending on the molecular defect. The paper explains the molecular mechanisms (alterations in R/T state equilibrium, 2,3-DPG binding, and Bohr effect), clinical presentation, diagnosis through oxygen dissociation curve measurement, and treatment approaches.

Traits studied:ErythrocytosisHigh oxygen affinity hemoglobinPolycythemia

About HBB

The alpha (HBA) and beta (HBB) loci determine the structure of the 2 types of polypeptide chains in adult hemoglobin, Hb A. The normal adult hemoglobin tetramer consists of two alpha chains and two beta chains. Mutant beta globin causes sickle cell anemia. Absence of beta chain causes beta-zero-thalassemia. Reduced amounts of detectable beta globin causes beta-plus-thalassemia. The order of the genes in the beta-globin cluster is 5'-epsilon -- gamma-G -- gamma-A -- delta -- beta--3'. [provided by RefSeq, Jul 2008]

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Gene information from NCBI Gene. Variant classifications from ClinVar.

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