Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Prevention |
RCV004529333 | SCV004103304 | pathogenic | MECP2-related disorder | 2023-03-28 | criteria provided, single submitter | clinical testing | The MECP2 c.902delT variant is predicted to result in a frameshift and premature protein termination (p.Leu301Profs*20). To our knowledge, this variant has not been reported in the literature or in a large population database (http://gnomad.broadinstitute.org), indicating it is rare. Frameshift variants in MECP2 are expected to be pathogenic, and several examples immediately flanking this position have been associated with Rett syndrome (see for example, Philippe et al. 2006. PubMed ID: 16473305). This variant is interpreted as pathogenic. |