Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001060589 | SCV001225289 | uncertain significance | not provided | 2021-08-28 | criteria provided, single submitter | clinical testing | This sequence change replaces glutamine with arginine at codon 984 of the ELP1 protein (p.Gln984Arg). The glutamine residue is weakly conserved and there is a small physicochemical difference between glutamine and arginine. This variant is present in population databases (rs371186754, ExAC 0.001%). This variant has not been reported in the literature in individuals affected with ELP1-related conditions. 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 tolerated. 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. |
St. |
RCV003325222 | SCV004031124 | uncertain significance | Medulloblastoma | 2023-08-17 | criteria provided, single submitter | clinical testing | The ELP1 c.2951A>G (p.Gln984Arg) missense change has a maximum subpopulation frequency of 0.0065% in gnomAD v2.1.1 (https://gnomad.broadinstitute.org/). The in silico tool REVEL predicts a benign effect on protein function, but to our knowledge this prediction has not been confirmed by functional studies. To our knowledge, this variant has not been reported in individuals with ELP1-associated disorders. In summary, the evidence currently available is insufficient to determine the clinical significance of this variant. It has therefore been classified as of uncertain significance. |
Natera, |
RCV001832542 | SCV002082055 | uncertain significance | Familial dysautonomia | 2020-08-05 | no assertion criteria provided | clinical testing |