Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
UNC Molecular Genetics Laboratory, |
RCV001249830 | SCV001423842 | uncertain significance | Aicardi-Goutieres syndrome 7 | criteria provided, single submitter | research | The IFIH1 c.2759T>C missense variant changes a single, evolutionarily well-conserved amino acid in the protein coding region from an isoleucine to a threonine. This variant has not been reported in control population databases or patient databases. | |
Labcorp Genetics |
RCV001879765 | SCV002278277 | uncertain significance | Singleton-Merten syndrome 1; Aicardi-Goutieres syndrome 7 | 2023-09-28 | criteria provided, single submitter | clinical testing | 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. Advanced modeling performed at Invitae incorporating data from internal and/or published experimental studies (Invitae) indicates that this missense variant is not expected to disrupt IFIH1 function. ClinVar contains an entry for this variant (Variation ID: 973334). This variant has not been reported in the literature in individuals affected with IFIH1-related conditions. This variant is not present in population databases (gnomAD no frequency). This sequence change replaces isoleucine, which is neutral and non-polar, with threonine, which is neutral and polar, at codon 920 of the IFIH1 protein (p.Ile920Thr). |
Ambry Genetics | RCV004035294 | SCV004887726 | uncertain significance | Inborn genetic diseases | 2023-12-16 | criteria provided, single submitter | clinical testing | The c.2759T>C (p.I920T) alteration is located in exon 14 (coding exon 14) of the IFIH1 gene. This alteration results from a T to C substitution at nucleotide position 2759, causing the isoleucine (I) at amino acid position 920 to be replaced by a threonine (T). Based on insufficient or conflicting evidence, the clinical significance of this alteration remains unclear. |