Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Eurofins Ntd Llc |
RCV000177261 | SCV000229104 | uncertain significance | not provided | 2015-04-09 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV004700539 | SCV005203654 | pathogenic | Allan-Herndon-Dudley syndrome | 2024-07-05 | criteria provided, single submitter | clinical testing | Variant summary: SLC16A2 c.604G>A (p.Gly202Arg) results in a non-conservative amino acid change in the encoded protein sequence. Five of five in-silico tools predict a damaging effect of the variant on protein function. The variant was absent in 182387 control chromosomes (gnomAD). c.604G>A has been reported in the literature in multiple individuals affected with Allan-Herndon-Dudley Syndrome (e.g. Choi_2018, Islam_2019, Groeneweg_2020). These data indicate that the variant is very likely to be associated with disease. At least one publication reports experimental evidence evaluating an impact on protein function, finding that the variant results in 10%-<30% of normal T3 uptake activity (Islam_2019). The following publications have been ascertained in the context of this evaluation (PMID: 30497070, 30369548, 32559475). ClinVar contains an entry for this variant (Variation ID: 196440). Based on the evidence outlined above, the variant was classified as pathogenic. |