Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Seattle Children's Hospital Molecular Genetics Laboratory, |
RCV001526668 | SCV001656298 | uncertain significance | Maturity onset diabetes mellitus in young | 2018-04-23 | criteria provided, single submitter | clinical testing | The c.82G>A (p.Glu28Lys) variant is predicted to substitute the glutamate at amino acid position 28 with a lysine. In silico tools have conflicting predictions as to the impact of this missense change on protein function. This alteration has not been observed by our lab and is not reported in disease databases (ClinVar) or in control populations (GnomAD). This variant has been reported in one individual with a clinical diagnosis of MODY but the clinical details of that family are limited (PMID: 19790256). Per ACMG criteria, this alteration is interpreted as a variant of uncertain clinical significance. |
Labcorp Genetics |
RCV005094714 | SCV005835032 | uncertain significance | not provided | 2024-08-10 | criteria provided, single submitter | clinical testing | This sequence change replaces glutamic acid, which is acidic and polar, with lysine, which is basic and polar, at codon 28 of the GCK protein (p.Glu28Lys). This variant is not present in population databases (gnomAD no frequency). This missense change has been observed in individual(s) with clinical features of GCK-related conditions (PMID: 19790256, 33046911). ClinVar contains an entry for this variant (Variation ID: 1172675). Advanced modeling of protein sequence and biophysical properties (such as structural, functional, and spatial information, amino acid conservation, physicochemical variation, residue mobility, and thermodynamic stability) performed at Invitae indicates that this missense variant is not expected to disrupt GCK protein function with a negative predictive value of 80%. 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. |