Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Diagnostics Services |
RCV004723644 | SCV005326532 | likely pathogenic | Neurodevelopmental disorder with regression, abnormal movements, loss of speech, and seizures | 2024-09-12 | criteria provided, single submitter | clinical testing | The c.587dup variant is not present in publicly available population databases like 1000 Genomes, EVS, ExAC, gnomAD, Indian Exome Database or our in-house exome database. This variant has neither been published in the literature in individuals affected with IRF2BPL-related conditions nor reported to the ClinVar, HGMD or OMIM databases, in any affected individuals. In-silico pathogenicity prediction programs likeMutationTaster2, CADD, Varsome, Franklin etc. predicted these variants to be likely deleterious. This variant causes frameshift at the 197th 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 a truncated protein or cause nonsense mediated decay of the mRNA. |