Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000541202 | SCV000628602 | pathogenic | Neurofibromatosis, type 1 | 2023-07-12 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. Studies have shown that disruption of this splice site results in activation of cryptic splice sites and introduces a premature termination codon (PMID: 10862084; Invitae). The resulting mRNA is expected to undergo nonsense-mediated decay. ClinVar contains an entry for this variant (Variation ID: 457705). Disruption of this splice site has been observed in individual(s) with neurofibromatosis type I (PMID: 10712197, 10862084). This variant is not present in population databases (gnomAD no frequency). This sequence change affects an acceptor splice site in intron 33 of the NF1 gene. RNA analysis indicates that disruption of this splice site induces altered splicing and may result in an absent or disrupted protein product. |
Genomics England Pilot Project, |
RCV001542605 | SCV001760400 | pathogenic | Neurofibromatosis-Noonan syndrome | no assertion criteria provided | clinical testing |