Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Diagnostics Services |
RCV004586409 | SCV005077766 | likely pathogenic | Neurodevelopmental disorder with regression, abnormal movements, loss of speech, and seizures | 2024-04-18 | criteria provided, single submitter | clinical testing | The c.320_321insGC variant is not present in publicly available population databases like 1000 Genomes, EVS, ExAC, Indian Exome Database or our in-house exome database. The variant present is present in the gnomAD database at a low frequency. This variant has neither been published in the literature with IRF2BPL-related conditions nor reported to the clinical databases like ClinVar, Human Genome Mutation Database (HGMD) or OMIM, in any affected individuals. In-silico pathogenicity prediction programs like MutationTaster2, CADD, Varsome, Franklin etc predicted the c.320_321insGC variant to be likely deleterious. This variant causes frameshift at the 108th amino acid position of the wild-type transcript which creates a premature translational stop signal at the altered transcript that may either result in translation of truncated protein or cause nonsense mediated decay of the mRNA. |