Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Groupe Hospitalier Pitie Salpetriere, |
RCV000850165 | SCV000930622 | pathogenic | Seizure; Intellectual disability; Moderate global developmental delay | 2019-08-05 | criteria provided, single submitter | clinical testing | A first LoF variant was reported in one family with autosomal recessive intellectual disabilities (Najmabadi et al, 2011). The NM_001959.3:c.383C>A, NP_001950.1:p.(Ser128*) variant segregates in the sibship, and is absent in general population alleles in gnomAD ascertained by July 2019. It is a LoF variant in a gene where another LoF variant has been supposed to induce similar symptoms. Moreover, there are multiple lines of computational evidence that support a deleterious effect on the gene or gene product. |