Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Human Genetics, |
RCV003330048 | SCV004034083 | likely pathogenic | Coffin-Siris syndrome 1 | 2023-09-12 | criteria provided, single submitter | clinical testing | The variant c.5983del (p.(Ser1995Valfs*62)) in exon 20 of the ARID1B-gene is not found in the gnomAD database, it creates a frame shift starting at codon Ser1995. The new reading frame ends in a STOP codon at position 62. Frameshift variants leading to a loss of function of ARID1B protein are a known mechanism of disease. This variant was found to be de novo in a patient with suspected Coffin-Siris Syndrom (PMID: 33768696, indicated here as NM_020732.3:c.5734del p.(Ser1912Valfs*62)). ACMG criteria used for classification: PVS1_str, PM2_supp, PP5. |