Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002008674 | SCV002277461 | pathogenic | Neurofibromatosis, type 1 | 2023-10-03 | criteria provided, single submitter | clinical testing | This variant, c.3809_3838del, results in the deletion of 10 amino acid(s) of the NF1 protein (p.Ser1270_Ser1279del), but otherwise preserves the integrity of the reading frame. This variant is not present in population databases (gnomAD no frequency). This variant has been observed in individual(s) with neurofibromatosis type 1 (Invitae). ClinVar contains an entry for this variant (Variation ID: 1485454). This variant disrupts a region of the NF1 protein in which other variant(s) (p.Arg1276Gly) have been determined to be pathogenic (PMID: 8628317, 15060124, 31595648). This suggests that this is a clinically significant region of the protein, and that variants that disrupt it are likely to be disease-causing. For these reasons, this variant has been classified as Pathogenic. |