Total submissions: 9
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Mendelics | RCV000083696 | SCV001139855 | uncertain significance | Familial Mediterranean fever | 2019-05-28 | criteria provided, single submitter | clinical testing | |
Gene |
RCV001562614 | SCV001785405 | uncertain significance | not provided | 2022-06-21 | criteria provided, single submitter | clinical testing | Reported in published literature in individuals from a family with an autosomal dominant periodic inflammatory disorder with renal AA amyloidosis and was found to exclusively co-segregate with the disease in all individuals tested (Aldea et al., 2004); In silico analysis, which includes protein predictors and evolutionary conservation, supports that this variant does not alter protein structure/function; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 19479870, 22614345, 18328141, 29260407, 23844200, 23031807, 27225717, 15024744, 14679589) |
Labcorp Genetics |
RCV000083696 | SCV002300876 | uncertain significance | Familial Mediterranean fever | 2022-08-08 | criteria provided, single submitter | clinical testing | 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. Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be tolerated. ClinVar contains an entry for this variant (Variation ID: 2557). This missense change has been observed in individual(s) with familial Mediterranean fever (PMID: 14679589). This variant is not present in population databases (gnomAD no frequency). This sequence change replaces histidine, which is basic and polar, with tyrosine, which is neutral and polar, at codon 478 of the MEFV protein (p.His478Tyr). |
Ce |
RCV001562614 | SCV002563301 | uncertain significance | not provided | 2022-07-01 | criteria provided, single submitter | clinical testing | MEFV: PM2, PP1:Moderate, PP4, BP4 |
Genome- |
RCV000083696 | SCV003802130 | uncertain significance | Familial Mediterranean fever | 2023-02-08 | criteria provided, single submitter | clinical testing | |
Genome- |
RCV000002666 | SCV003802131 | likely benign | Familial Mediterranean fever, autosomal dominant | 2023-02-08 | criteria provided, single submitter | clinical testing | |
Genome- |
RCV003125827 | SCV003802132 | likely benign | Acute febrile neutrophilic dermatosis | 2023-02-08 | criteria provided, single submitter | clinical testing | |
OMIM | RCV000002666 | SCV000022824 | pathogenic | Familial Mediterranean fever, autosomal dominant | 2004-01-01 | no assertion criteria provided | literature only | |
Unité médicale des maladies autoinflammatoires, |
RCV000083696 | SCV000115784 | not provided | Familial Mediterranean fever | no assertion provided | not provided |