Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV002037124 | SCV002111138 | likely pathogenic | Neurofibromatosis, type 1 | 2021-10-25 | criteria provided, single submitter | clinical testing | In summary, the currently available evidence indicates that the variant is pathogenic, but additional data are needed to prove that conclusively. Therefore, this variant has been classified as Likely Pathogenic. 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) performed at Invitae indicates that this missense variant is expected to disrupt NF1 protein function. This missense change has been observed in individual(s) with neurofibromatosis type 1 (PMID: 25074460). This variant is not present in population databases (ExAC no frequency). This sequence change replaces threonine with proline at codon 1343 of the NF1 protein (p.Thr1343Pro). The threonine residue is moderately conserved and there is a small physicochemical difference between threonine and proline. |