Total submissions: 4
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Clin |
RCV001843397 | SCV002102527 | uncertain significance | Monogenic diabetes | 2024-01-18 | reviewed by expert panel | curation | The c.-258A>G variant in the HNF1 homeobox A gene, HNF1A, is located in the promoter of NM_000545.8. This variant is located in the promoter of NM_000545.8. This variant is located at the HNF1A binding site (c.-238 to c.-259) of HNF1A, which is defined as critical for the protein’s function by the ClinGen MDEP (PM1_Supporting). Additionally, this variant was identified in an individual with a clinical history highly specific for HNF1A-MODY (MODY probability calculator result >50%, negative genetic testing for HNF4A) (PP4; internal lab contributor). In summary, c.-258A>G meets the criteria to be classified as a variant of uncertain significance for monogenic diabetes. ACMG/AMP criteria applied, as specified by the ClinGen MDEP (specification version 2.1.0, approved 8/11/2023): PM1_Supporting, PP4. |
Fulgent Genetics, |
RCV002482386 | SCV002783164 | uncertain significance | Diabetes mellitus type 1; Type 1 diabetes mellitus 20; Maturity-onset diabetes of the young type 3; Type 2 diabetes mellitus; Hepatic adenomas, familial; Nonpapillary renal cell carcinoma | 2022-01-04 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV002545231 | SCV003471765 | benign | not provided | 2024-10-23 | criteria provided, single submitter | clinical testing | |
Ce |
RCV002545231 | SCV005042043 | uncertain significance | not provided | 2024-04-01 | criteria provided, single submitter | clinical testing |