Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000796043 | SCV000935534 | uncertain significance | Androgen resistance syndrome; Kennedy disease | 2018-12-11 | criteria provided, single submitter | clinical testing | This sequence change results in a frameshift in the AR gene (p.His918Thrfs*26). While this is not anticipated to result in nonsense mediated decay, it is expected to disrupt the last 3 amino acids of the AR protein and extend the protein by an additional 8 amino acids. This variant is not present in population databases (ExAC no frequency). This variant has not been reported in the literature in individuals with AR-related disease. The observation of one or more missense substitutions at or downstream of this variant (p.His918Arg) in affected individuals suggests that this may be a clinically significant region of the AR protein (PMID: 10690872, 18577874). 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. |