Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
ARUP Laboratories, |
RCV001811181 | SCV001157414 | likely benign | not provided | 2022-04-01 | criteria provided, single submitter | clinical testing | |
Quest Diagnostics Nichols Institute San Juan Capistrano | RCV001811181 | SCV004219816 | uncertain significance | not provided | 2023-05-03 | criteria provided, single submitter | clinical testing | The frequency of this variant in the general population, 0.00015 (5/34326 chromosomes, http://gnomad.broadinstitute.org), is uninformative in assessment of its pathogenicity. In the published literature, the variant has been reported in a family with normal hematological parameters and was initially described as being stable (PMID: 3839772 (1985)). The variant was also predicted to be unstable and have a potential negative effect on oxygen binding based on analysis of the protein structure (PMID: 31553106 (2020)). Analysis of this variant using bioinformatics tools for the prediction of the effect of amino acid changes on protein structure and function yielded predictions that this variant is benign. Based on the available information, we are unable to determine the clinical significance of this variant. |
OMIM | RCV000017173 | SCV000037445 | other | HEMOGLOBIN TWIN PEAKS | 2016-07-20 | no assertion criteria provided | literature only | |
Prevention |
RCV004755739 | SCV005349401 | uncertain significance | HBA1-related disorder | 2024-05-14 | no assertion criteria provided | clinical testing | The HBA1 c.341T>A variant is predicted to result in the amino acid substitution p.Leu114His. To our knowledge, this variant has not been reported in the literature in association with disease. This variant is reported in 0.015% of alleles in individuals of Latino descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |