Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001921050 | SCV002190084 | uncertain significance | Cenani-Lenz syndactyly syndrome; Sclerosteosis 2; Congenital myasthenic syndrome 17 | 2022-08-09 | 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. ClinVar contains an entry for this variant (Variation ID: 1415683). This variant has not been reported in the literature in individuals affected with LRP4-related conditions. This variant is present in population databases (rs752315031, gnomAD 0.006%). This sequence change replaces arginine, which is basic and polar, with histidine, which is basic and polar, at codon 916 of the LRP4 protein (p.Arg916His). |
Fulgent Genetics, |
RCV001921050 | SCV002794229 | uncertain significance | Cenani-Lenz syndactyly syndrome; Sclerosteosis 2; Congenital myasthenic syndrome 17 | 2022-01-29 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV004738438 | SCV005362302 | uncertain significance | LRP4-related disorder | 2024-03-05 | no assertion criteria provided | clinical testing | The LRP4 c.2747G>A variant is predicted to result in the amino acid substitution p.Arg916His. To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.0058% 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. |