Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV001008181 | SCV001167947 | pathogenic | not provided | 2019-03-04 | criteria provided, single submitter | clinical testing | The c.1243delG variant in the ARID1B gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The c.1243delG variant causes a frameshift starting with codon Alanine 415, changes this amino acid to a Proline residue, and creates a premature Stop codon at position 15 of the new reading frame, denoted p.Ala415ProfsX15. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The c.1243delG variant is not observed in large population cohorts (Lek et al., 2016). We interpret c.1243delG as a pathogenic variant. |
Institute of Human Genetics, |
RCV001293361 | SCV001481814 | pathogenic | Intellectual disability | 2021-02-25 | criteria provided, single submitter | clinical testing | The variant chr6-157100301-CG-C, ARID1B(NM_017519.3):c.1492delG,p.(Ala498Profs*15) was identified in an individual with NDD. Inheritance was de novo (heterozygous). The variant was reviewed according to current ACMG recommendations and classified as Pathogenic (criteria: PVS1_VeryStrong, PS2_Moderate, PM2_Supporting). |
Genome- |
RCV001807376 | SCV002054263 | pathogenic | Coffin-Siris syndrome 1 | 2021-07-15 | criteria provided, single submitter | clinical testing |