Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Servicio de Genética Del Instituto Nacional de Salud Del Niño, |
RCV004794767 | SCV005414512 | likely pathogenic | Rubinstein-Taybi syndrome due to EP300 haploinsufficiency | 2024-11-18 | criteria provided, single submitter | clinical testing | The variant NM_001429.4:c.2959delC (p.Pro987Glnfs*33) results in a deletion that causes a frameshift and a premature stop codon 33 amino acids downstream. This is predicted to lead to nonsense-mediated decay (NMD) or a truncated protein. According to ACMG/AMP guidelines, this variant meets the criteria for PVS1 and PM2, supporting its classification as likely pathogenic |