Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001220956 | SCV001392971 | uncertain significance | Neurofibromatosis, type 1 | 2022-11-12 | 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 sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) has been performed at Invitae for this missense variant, however the output from this modeling did not meet the statistical confidence thresholds required to predict the impact of this variant on NF1 protein function. ClinVar contains an entry for this variant (Variation ID: 949487). This missense change has been observed in individual(s) with clinical features of neurofibromatosis type 1 (PMID: 18546366). This variant is not present in population databases (gnomAD no frequency). This sequence change replaces asparagine, which is neutral and polar, with serine, which is neutral and polar, at codon 412 of the NF1 protein (p.Asn412Ser). |
Medical Genetics, |
RCV001220956 | SCV002567772 | likely pathogenic | Neurofibromatosis, type 1 | 2022-08-17 | criteria provided, single submitter | clinical testing | |
Gene |
RCV002466637 | SCV002762164 | likely pathogenic | not provided | 2022-06-07 | criteria provided, single submitter | clinical testing | Demonstrated to create a new splice site resulting in an in-frame loss of Val411_Asn420 in patient RNA (Pros et al., 2008); Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 18546366) |