Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Institute of Medical Genetics and Applied Genomics, |
RCV001268743 | SCV001447891 | pathogenic | not provided | 2020-10-23 | criteria provided, single submitter | clinical testing | |
Laboratory of Medical Genetics, |
RCV002464433 | SCV002760156 | pathogenic | Coffin-Siris syndrome 1 | 2022-11-29 | criteria provided, single submitter | research | |
Ambry Genetics | RCV004960667 | SCV005480798 | pathogenic | Inborn genetic diseases | 2024-07-10 | criteria provided, single submitter | clinical testing | The c.5457G>A (p.W1819*) alteration, located in exon 20 (coding exon 20) of the ARID1B gene, consists of a G to A substitution at nucleotide position 5457. This changes the amino acid from a tryptophan (W) to a stop codon at amino acid position 1819. This alteration occurs at the 3' terminus of the ARID1B gene, is not expected to trigger nonsense-mediated mRNA decay, and impacts the last 19.2% of the protein. However, premature stop codons are typically deleterious in nature, the impacted region is critical for protein function, and a significant portion of the protein is affected. This variant was not reported in population-based cohorts in the Genome Aggregation Database (gnomAD). This variant was reported in an individual with features consistent with ARID1B-related Coffin-Siris syndrome (Wieczorek, 2013). Based on the available evidence, this alteration is classified as pathogenic. |