Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000813988 | SCV000954375 | uncertain significance | Hyper-IgE recurrent infection syndrome 1, autosomal dominant; STAT3 gain of function | 2018-08-23 | criteria provided, single submitter | clinical testing | This sequence change replaces serine with alanine at codon 465 of the STAT3 protein (p.Ser465Ala). The serine residue is highly conserved and there is a moderate physicochemical difference between serine and alanine. This variant is not present in population databases (ExAC no frequency). This variant has been observed in an individual affected with hyper-IgE syndrome (PMID: 17881745). Experimental studies have shown that this missense change affects normal STAT3 protein function (PMID: 26384563). 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. |