Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Genetic Services Laboratory, |
RCV001820556 | SCV002070144 | uncertain significance | not specified | 2020-02-04 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV002478058 | SCV002788299 | uncertain significance | Diabetes mellitus, transient neonatal, 2; Hyperinsulinemic hypoglycemia, familial, 1; Leucine-induced hypoglycemia; Type 2 diabetes mellitus; Diabetes mellitus, permanent neonatal 3 | 2021-09-30 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV003107857 | SCV003783757 | uncertain significance | not provided | 2022-10-17 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with glutamine, which is neutral and polar, at codon 933 of the ABCC8 protein (p.Arg933Gln). This variant is present in population databases (rs745591375, gnomAD 0.007%). This missense change has been observed in individual(s) with clinical features of hyperinsulinism (PMID: 32027066). ClinVar contains an entry for this variant (Variation ID: 1337542). 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. 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. |