Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Medical Genetics, |
RCV000497216 | SCV000588728 | likely pathogenic | Neurofibromatosis, type 1 | 2022-08-17 | criteria provided, single submitter | clinical testing | |
Greenwood Genetic Center Diagnostic Laboratories, |
RCV000497216 | SCV002061881 | likely pathogenic | Neurofibromatosis, type 1 | 2021-06-04 | criteria provided, single submitter | clinical testing | PS4_Supporting, PP2, PP3, PM2, PM6 |
Labcorp Genetics |
RCV000497216 | SCV004539027 | likely pathogenic | Neurofibromatosis, type 1 | 2023-07-28 | 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. ClinVar contains an entry for this variant (Variation ID: 431592). This missense change has been observed in individual(s) with neurofibromatosis type I (PMID: 28961165; Invitae). This variant is not present in population databases (gnomAD no frequency). This sequence change replaces leucine, which is neutral and non-polar, with phenylalanine, which is neutral and non-polar, at codon 529 of the NF1 protein (p.Leu529Phe). |
Ambry Genetics | RCV004559129 | SCV005048110 | uncertain significance | Hereditary cancer-predisposing syndrome; Cardiovascular phenotype | 2024-02-09 | criteria provided, single submitter | clinical testing | The p.L529F variant (also known as c.1585C>T), located in coding exon 14 of the NF1 gene, results from a C to T substitution at nucleotide position 1585. The leucine at codon 529 is replaced by phenylalanine, an amino acid with highly similar properties. This alteration was identified in an individual with a clinical diagnosis of neurofibromatosis type 1 (NF1) (Bonatti F et al. Int J Mol Sci, 2017 Sep;18:). This amino acid position is highly conserved in available vertebrate species. In addition, this alteration is predicted to be deleterious by in silico analysis. Based on the available evidence, the clinical significance of this alteration remains unclear. |