Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Foundation for Research in Genetics and Endocrinology, |
RCV003486000 | SCV004232640 | pathogenic | Glycogen storage disease, type II | 2024-01-25 | criteria provided, single submitter | clinical testing | A homozygous 2 base pair deletion in exon 20 of the GAA gene that results in a frameshift and premature truncation of the protein 72 amino acids downstream to codon 945 was detected. The observed variant c.2834_2835 del (p.Glu945Alafs*72) has not been reported in the gnomAD database. The in silico prediction of the variant is damaging by MutationTaster2. In summary, the variant meets our criteria to be classified as pathogenic. |