Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001911417 | SCV002174481 | uncertain significance | not provided | 2024-01-12 | criteria provided, single submitter | clinical testing | This sequence change replaces arginine, which is basic and polar, with histidine, which is basic and polar, at codon 974 of the DUOX2 protein (p.Arg974His). This variant also falls at the last nucleotide of exon 22, which is part of the consensus splice site for this exon. This variant is present in population databases (rs778216481, gnomAD 0.1%). This missense change has been observed in individual(s) with congenital hypothyroidism (PMID: 29650690, 32425884, 33631011, 34539567). In at least one individual the data is consistent with being in trans (on the opposite chromosome) from a pathogenic variant. ClinVar contains an entry for this variant (Variation ID: 1400637). An algorithm developed to predict the effect of missense changes on protein structure and function (PolyPhen-2) suggests that this variant is likely to be tolerated. Variants that disrupt the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). Algorithms developed to predict the effect of sequence changes on RNA splicing suggest that this variant may disrupt the consensus splice site. 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. |
Fulgent Genetics, |
RCV002490196 | SCV002791661 | uncertain significance | Thyroid dyshormonogenesis 6 | 2022-04-21 | criteria provided, single submitter | clinical testing | |
Gene |
RCV001911417 | SCV005328234 | uncertain significance | not provided | 2023-08-30 | criteria provided, single submitter | clinical testing | Identified in unrelated individuals with congenital hypothyroidism who were also heterozygous for other variants in DUOX2; the phase of these variants was not reported (Sun F et al., 2018); In silico analysis supports that this missense variant has a deleterious effect on protein structure/function; In silico analysis supports that this missense variant does not alter protein structure/function; This variant is associated with the following publications: (PMID: 17576681, 33631011, 34539567, 33644218, 32425884, 29650690) |