Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002843077 | SCV003211888 | uncertain significance | Bloom syndrome | 2022-06-30 | criteria provided, single submitter | clinical testing | This variant is also known as G3181T at codon 1036. This sequence change replaces valine, which is neutral and non-polar, with phenylalanine, which is neutral and non-polar, at codon 1061 of the BLM protein (p.Val1061Phe). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with Bloom syndrome (PMID: 9285778). 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 C0"). Studies have shown that this missense change alters BLM gene expression (PMID: 9285778). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may create or strengthen a splice site. 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. |