Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001768896 | SCV001993660 | uncertain significance | not provided | 2019-05-09 | criteria provided, single submitter | clinical testing | Not observed at a significant frequency in large population cohorts (Lek et al., 2016); Has not been previously published as pathogenic or benign to our knowledge; In-silico analysis, which includes splice predictors and evolutionary conservation, is inconclusive as to whether the variant alters gene splicing. In the absence of RNA/functional studies, the actual effect of this sequence change is unknown. |
Labcorp Genetics |
RCV002540258 | SCV003297087 | uncertain significance | Hyperphosphatasia with intellectual disability syndrome 2 | 2022-08-13 | 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. Variants that disrupt the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant is not likely to affect RNA splicing. ClinVar contains an entry for this variant (Variation ID: 1305689). This variant has not been reported in the literature in individuals affected with PIGO-related conditions. This variant is present in population databases (rs746026821, gnomAD 0.003%). This sequence change falls in intron 8 of the PIGO gene. It does not directly change the encoded amino acid sequence of the PIGO protein. It affects a nucleotide within the consensus splice site. |