Total submissions: 6
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001769689 | SCV002004585 | uncertain significance | not provided | 2024-07-24 | criteria provided, single submitter | clinical testing | Published functional studies demonstrate a damaging effect on promoter activity (PMID: 20133622); Nucleotide is not conserved across species and the substitution has no predicted effect on splicing; Also known as c.-332C>G; This variant is associated with the following publications: (PMID: 20133622, 30977832) |
Genetic Services Laboratory, |
RCV001821999 | SCV002067365 | uncertain significance | not specified | 2019-08-27 | criteria provided, single submitter | clinical testing | |
MGZ Medical Genetics Center | RCV002290758 | SCV002580772 | likely pathogenic | Maturity-onset diabetes of the young type 10 | 2022-04-28 | criteria provided, single submitter | clinical testing | |
Fulgent Genetics, |
RCV002506806 | SCV002816827 | uncertain significance | Type 1 diabetes mellitus 2; Maturity-onset diabetes of the young type 10; Hyperproinsulinemia; Diabetes mellitus, permanent neonatal 4 | 2022-04-10 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV001769689 | SCV003439677 | benign | not provided | 2023-03-14 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV001821999 | SCV004813500 | likely benign | not specified | 2024-02-06 | criteria provided, single submitter | clinical testing | Variant summary: INS c.-153C>G is located in the untranscribed region upstream of the INS gene region. Consensus agreement among computation tools predict no significant impact on normal splicing. However, these predictions have yet to be confirmed by functional studies. The variant allele was found at a frequency of 0.0002 in 308020 control chromosomes, predominantly at a frequency of 0.0028 within the East Asian subpopulation in the gnomAD database. The observed variant frequency within East Asian control individuals in the gnomAD database is approximately 3 fold of the estimated maximal expected allele frequency for a pathogenic variant in INS causing Neonatal Diabetes Mellitus phenotype (recessive 0.001), strongly suggesting that the variant is a benign polymorphism found primarily in populations of East Asian origin. c.-153C>G has been reported in the literature in individuals affected with Neonatal Diabetes Mellitus or monogenic diabetes. These report(s) do not provide unequivocal conclusions about association of the variant with Neonatal Diabetes Mellitus. At least one publication reports experimental evidence evaluating an impact on promoter activity. The most pronounced variant effect results in about 35% of normal activity. The following publications have been ascertained in the context of this evaluation (PMID: 20133622, 30977832). ClinVar contains an entry for this variant (Variation ID: 1317682). Based on the evidence outlined above, the variant was classified as likely benign. |