Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001246011 | SCV001419338 | uncertain significance | not provided | 2021-09-02 | criteria provided, single submitter | clinical testing | This sequence change replaces isoleucine with threonine at codon 936 of the ELP1 protein (p.Ile936Thr). The isoleucine residue is highly conserved and there is a moderate physicochemical difference between isoleucine and threonine. The frequency data for this variant in the population databases is considered unreliable, as metrics indicate poor data quality at this position in the ExAC database. This variant has not been reported in the literature in individuals affected with ELP1-related conditions. Algorithms developed to predict the effect of missense changes on protein structure and function (SIFT, PolyPhen-2, Align-GVGD) all suggest that this variant is likely to be disruptive. 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. |
Ambry Genetics | RCV004034846 | SCV002755541 | uncertain significance | not specified | 2021-06-11 | criteria provided, single submitter | clinical testing | The p.I936T variant (also known as c.2807T>C), located in coding exon 25 of the IKBKAP gene, results from a T to C substitution at nucleotide position 2807. The isoleucine at codon 936 is replaced by threonine, an amino acid with similar properties. This amino acid position is highly conserved in available vertebrate species. In addition, this alteration is predicted to be deleterious by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this alteration remains unclear. |
Natera, |
RCV001835255 | SCV002082064 | uncertain significance | Familial dysautonomia | 2020-08-04 | no assertion criteria provided | clinical testing |