Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Diagnostics Services |
RCV004723637 | SCV005326519 | likely pathogenic | Intellectual disability-feeding difficulties-developmental delay-microcephaly syndrome | 2024-08-14 | criteria provided, single submitter | clinical testing | The c.1342C>T variant is not present in publicly available population databases like 1000 Genomes, EVS, Indian Exome Database or our in-house exome database. This variant is present in ExAC and gnomAD at low frequencies. This variant has been previously reported in an individual with nodular medulloblastoma [PMID: 30419952]. In-silico pathogenicity prediction programs like MutationTaster2021, CADD, Franklin, Varsome etc predicted this variant to be likely deleterious. This variant creates a premature translational stop signal at the 448th amino acid position of the wild-type transcript that may either result in translation of a truncated protein or cause nonsense mediated decay of the mRNA. This variant is located in a mutational hotspot region of the gene and a missense variant in the same position (Arg448Gln) has been previously reported in patients affected with neurodevelopmental disorders [PMID: 31239556, 33004838]. |