Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV002227398 | SCV002506509 | likely pathogenic | Intellectual disability-microcephaly-strabismus-behavioral abnormalities syndrome | 2022-05-05 | criteria provided, single submitter | clinical testing | The variant c.2545+2del (p.?) is located in the donor splice site of intron 17 of the POGZ-gene, it affects a canonical splice site and it is not found in the gnomAD database. In silico programs predict a significant impact on POGZ-RNA splicing (varSEAK SSP). This variant was found in a patient, who was referred to our institute due to speech developemental delay and microcephaly. ACMG criteria used for classification: PVS1, PM2. |