Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003093631 | SCV003470748 | uncertain significance | Bloom syndrome | 2022-07-29 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. Algorithms developed to predict the effect of missense changes on protein structure and function are either unavailable or do not agree on the potential impact of this missense change (SIFT: "Deleterious"; PolyPhen-2: "Probably Damaging"; Align-GVGD: "Class C15"). This variant has not been reported in the literature in individuals affected with BLM-related conditions. This variant is not present in population databases (gnomAD no frequency). This sequence change replaces serine, which is neutral and polar, with leucine, which is neutral and non-polar, at codon 1138 of the BLM protein (p.Ser1138Leu). |
Ambry Genetics | RCV005310900 | SCV005965761 | uncertain significance | Hereditary cancer-predisposing syndrome | 2025-02-01 | criteria provided, single submitter | clinical testing | The p.S1138L variant (also known as c.3413C>T), located in coding exon 17 of the BLM gene, results from a C to T substitution at nucleotide position 3413. The serine at codon 1138 is replaced by leucine, an amino acid with dissimilar properties. This amino acid position is conserved. In addition, this alteration is predicted to be deleterious by in silico analysis. Based on the available evidence, the clinical significance of this variant remains unclear. |