Total submissions: 4
| Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
|---|---|---|---|---|---|---|---|---|
| Gene |
RCV000254784 | SCV000321656 | pathogenic | not provided | 2025-03-09 | criteria provided, single submitter | clinical testing | Frameshift variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); Has not been previously published as pathogenic or benign to our knowledge |
| Labcorp Genetics |
RCV001389700 | SCV001591151 | pathogenic | Birt-Hogg-Dube syndrome | 2021-06-22 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. This variant has not been reported in the literature in individuals with FLCN-related conditions. ClinVar contains an entry for this variant (Variation ID: 265153). This variant is not present in population databases (ExAC no frequency). This sequence change creates a premature translational stop signal (p.Asn236Lysfs*6) in the FLCN gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in FLCN are known to be pathogenic (PMID: 15852235). |
| Ambry Genetics | RCV002365271 | SCV002664050 | pathogenic | Hereditary cancer-predisposing syndrome | 2022-10-24 | criteria provided, single submitter | clinical testing | The c.708delC pathogenic mutation, located in coding exon 4 of the FLCN gene, results from a deletion of one nucleotide at nucleotide position 708, causing a translational frameshift with a predicted alternate stop codon (p.N236Kfs*6). This alteration is expected to result in loss of function by premature protein truncation or nonsense-mediated mRNA decay. As such, this alteration is interpreted as a disease-causing mutation. |
| Myriad Genetics, |
RCV001389700 | SCV004189018 | pathogenic | Birt-Hogg-Dube syndrome | 2023-07-06 | criteria provided, single submitter | clinical testing | This variant is considered pathogenic. This variant creates a frameshift predicted to result in premature protein truncation. |