Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV001812287 | SCV001470867 | uncertain significance | not provided | 2019-09-27 | criteria provided, single submitter | clinical testing | The HBA1 c.150C>A; p.Ser50Arg variant (also known as Ser49Arg when numbered from the mature protein; rs1318437795) to our knowledge, is not reported in the medical literature or gene-specific databases. Two other variants that cause the same amino acid substitution in HBA2 (c.148A>C, c.150C>A) have been reported in heterozygous individuals without clinical symptoms, with the variant protein exhibiting normal stability and oxygen affinity (Tran Houangkeo 2016, HbVar link and references therein). The HBA1 c.150C>A variant is absent from general population databases (Exome Variant Server, Genome Aggregation Database), indicating it is not a common polymorphism. The serine at codon 50 is highly conserved, and computational analyses (SIFT, PolyPhen-2) predict that this variant is deleterious, although these are low confidence predictions. Due to limited information, the clinical significance of the HBA1 c.150C>A; p.Ser50Arg variant is uncertain at this time. References: HbVar link for HBA2 c.150C>A: http://globin.bx.psu.edu/cgi-bin/hbvar/query_vars3?mode=output&display_format=page&i=70 Tran Houangkeo TH et al. Hb Savaria [a49(CE7)Ser>Arg; HBA2: c.150C > A]: A New Case and Complete Description. Hemoglobin. 2016 Aug;40(4):267-9. |
Natera, |
RCV001830080 | SCV002093860 | uncertain significance | alpha Thalassemia | 2020-12-03 | no assertion criteria provided | clinical testing |