Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Diagnostics Services |
RCV003482203 | SCV004227986 | likely pathogenic | Neurodevelopmental disorder | 2024-01-05 | criteria provided, single submitter | clinical testing | Recently compound heterozygous variations in the DNAH14 gene (MIM*603341) have been reported to cause an autosomal recessive neurodevelopmental disorder characterized by seizures, global developmental delay, microcephaly and hypotonia [PMID: 35438214]. The c.3697C>T variant is not present in publicly available population databases like 1000 Genomes, EVS, Indian Exome Database or our in-house exome database. The variant is present in ExAC and gnomAD at low frequencies. This variant has neither been published in literature for DNAH14-related conditions nor reported to clinical databases like ClinVar, OMIM or HGMD, in any affected individuals. In-silico pathogenicity prediction programs like MutationTaster2, CADD, Varsome, Franklin, etc predicted this variant to be likely deleterious. This variant creates a premature translational stop codon at the 1233rd amino acid position of the transcript that may either result in translation of a truncated protein or cause nonsense mediated decay of the mRNA. |