Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Invitae | RCV001066615 | SCV001231630 | uncertain significance | Familial Mediterranean fever | 2022-06-23 | criteria provided, single submitter | clinical testing | This sequence change replaces leucine, which is neutral and non-polar, with valine, which is neutral and non-polar, at codon 508 of the MEFV protein (p.Leu508Val). This variant is present in population databases (rs199937453, gnomAD 0.05%). This variant has not been reported in the literature in individuals affected with MEFV-related conditions. ClinVar contains an entry for this variant (Variation ID: 860331). Algorithms developed to predict the effect of missense changes on protein structure and function output the following: SIFT: "Tolerated"; PolyPhen-2: "Possibly Damaging"; Align-GVGD: "Class C0". The valine amino acid residue is found in multiple mammalian species, which suggests that this missense change does not adversely affect protein function. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
ARUP Laboratories, |
RCV001811638 | SCV001473487 | uncertain significance | not provided | 2019-09-30 | criteria provided, single submitter | clinical testing | The MEFV c.1522C>G; p.Leu508Val variant (rs199937453), to our knowledge, is not reported in the medical literature or gene specific databases. This variant is found in the African population with an allele frequency of 0.048% (12/24970 alleles) in the Genome Aggregation Database. The leucine at codon 508 is highly conserved, and computational analyses (SIFT: tolerated, PolyPhen-2: probably damaging) predict conflicting effects of this variant on protein structure/function. Due to limited information, the clinical significance of the p.Leu508Val variant is uncertain at this time. |
Genome- |
RCV001066615 | SCV003802100 | uncertain significance | Familial Mediterranean fever | 2023-02-08 | criteria provided, single submitter | clinical testing | |
Genome- |
RCV001274313 | SCV003802102 | likely benign | Familial Mediterranean fever, autosomal dominant | 2023-02-08 | criteria provided, single submitter | clinical testing | |
Genome- |
RCV003127618 | SCV003802103 | likely benign | Acute febrile neutrophilic dermatosis | 2023-02-08 | criteria provided, single submitter | clinical testing | |
Natera, |
RCV001274313 | SCV001458301 | uncertain significance | Familial Mediterranean fever, autosomal dominant | 2020-06-09 | no assertion criteria provided | clinical testing |