Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001896344 | SCV002162697 | uncertain significance | Generalized epilepsy-paroxysmal dyskinesia syndrome | 2023-09-21 | criteria provided, single submitter | clinical testing | This variant is present in population databases (rs139370249, gnomAD 0.003%). 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. An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be disruptive. ClinVar contains an entry for this variant (Variation ID: 1392675). This missense change has been observed in individual(s) with clinical features of KCNMA1-related conditions (PMID: 29738522, 29933521). This sequence change replaces arginine, which is basic and polar, with tryptophan, which is neutral and slightly polar, at codon 1177 of the KCNMA1 protein (p.Arg1177Trp). |