Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV003110106 | SCV003762051 | likely pathogenic | not provided | 2023-01-24 | 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 |
Mayo Clinic Laboratories, |
RCV003110106 | SCV004225741 | likely pathogenic | not provided | 2023-03-15 | criteria provided, single submitter | clinical testing | PM2, PVS1 |
Labcorp Genetics |
RCV003110106 | SCV005696687 | pathogenic | not provided | 2024-11-27 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Trp16Glyfs*63) in the FGF3 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in FGF3 are known to be pathogenic (PMID: 18435799). This variant is present in population databases (no rsID available, gnomAD 0.007%). This premature translational stop signal has been observed in individual(s) with FGF3-related conditions (PMID: 36934406). ClinVar contains an entry for this variant (Variation ID: 2413091). For these reasons, this variant has been classified as Pathogenic. |
Prevention |
RCV004754962 | SCV005364752 | likely pathogenic | FGF3-related disorder | 2024-04-12 | no assertion criteria provided | clinical testing | The FGF3 c.45delC variant is predicted to result in a frameshift and premature protein termination (p.Gly15Glyfs*64). This variant has not been reported in the literature. This variant is reported in 0.0069% of alleles in individuals of Latino descent in gnomAD. Frameshift variants in FGF3 are expected to be pathogenic. This variant is interpreted as likely pathogenic. |