Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV003114195 | SCV003799698 | pathogenic | not provided | 2022-05-04 | criteria provided, single submitter | clinical testing | The Hb Buenos Aires variant (HBB: c.257T>C; p.Phe86Ser, also known as Hb Bryn Mawr, and as Phe85Ser when numbered from the mature protein, rs35693898, HbVar ID: 414) is reported in the literature in heterozygous individuals affected with hemolytic anemia (see HbVar and references therein). The is variant is reported as unstable with increased oxygen affinity (see HbVar). This variant is reported in ClinVar (Variation ID: 15122) and is absent from the Genome Aggregation Database, indicating it is not a common polymorphism. The phenylalanine at codon 86 is highly conserved, and computational analyses predict that this variant is deleterious (REVEL: 0.804). Based on available information, this variant is considered to be pathogenic. References: Link to HbVar database: https://globin.bx.psu.edu/hbvar/menu.html |
OMIM | RCV000016279 | SCV000036547 | pathogenic | Hemoglobinopathy | 1967-09-29 | no assertion criteria provided | literature only |