Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Provincial Medical Genetics Program of British Columbia, |
RCV002071023 | SCV002320816 | uncertain significance | Stickler syndrome type 2 | 2022-01-01 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002552346 | SCV002950292 | uncertain significance | not provided | 2022-02-27 | 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 (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be disruptive. This variant has not been reported in the literature in individuals affected with COL11A1-related conditions. The frequency data for this variant in the population databases is considered unreliable, as metrics indicate poor data quality at this position in the gnomAD database. This sequence change replaces proline, which is neutral and non-polar, with leucine, which is neutral and non-polar, at codon 496 of the COL11A1 protein (p.Pro496Leu). |