Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV002246186 | SCV002515884 | likely benign | Emery-Dreifuss muscular dystrophy 5, autosomal dominant | 2022-05-20 | criteria provided, single submitter | clinical testing | For the following reasons, we assess the SYNE2 sequence variant found to be "Likely benign": the mutation has not yet been described in the specialized literature or listed in the HGMD and ClinVar databases; a comparison with the gnomAD browser did not provide any evidence that this sequence change is a norm variant that can also be detected in non-affected individuals; the mutation occurs at a frequency of 0.0004% (gnomAD), and never in the homozygous state; Based on the finding that the unaffected mother of index patient is a carrier and assuming complete penetrance, the sequence variants in the SYNE2 gene most likely represent rare familial variants. |