Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Broad Center for Mendelian Genomics, |
RCV000785962 | SCV000924545 | likely pathogenic | Coffin-Siris syndrome 1 | 2018-06-15 | criteria provided, single submitter | research | The heterozygous p.Asn2158ThrfsTer2 variant was identified by our study in an individual with Coffin-Siris syndrome. Trio exome analysis showed this variant to be de novo. This variant was absent from large population studies. Loss of function of the ARID1B gene is an established disease mechanism in Coffin-Siris syndrome, and this is a loss of function variant. In summary, although additional studies are required to fully establish its pathogenicity, this variant is likely pathogenic. |