Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000674788 | SCV000800186 | likely benign | Nemaline myopathy 2 | 2018-05-24 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000674788 | SCV001226091 | uncertain significance | Nemaline myopathy 2 | 2022-07-06 | criteria provided, single submitter | clinical testing | This sequence change affects codon 8104 of the NEB mRNA. It is a 'silent' change, meaning that it does not change the encoded amino acid sequence of the NEB protein. This variant also falls at the last nucleotide of exon 171, which is part of the consensus splice site for this exon. This variant is present in population databases (rs763193315, gnomAD 0.002%). This variant has not been reported in the literature in individuals affected with NEB-related conditions. ClinVar contains an entry for this variant (Variation ID: 558507). 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 may disrupt the consensus splice site. 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. |
Prevention |
RCV004547845 | SCV004754781 | likely benign | NEB-related disorder | 2019-07-02 | no assertion criteria provided | clinical testing | This variant is classified as likely benign based on ACMG/AMP sequence variant interpretation guidelines (Richards et al. 2015 PMID: 25741868, with internal and published modifications). |