Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Mendelics | RCV000990683 | SCV001141706 | pathogenic | Duchenne muscular dystrophy | 2024-03-15 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000990683 | SCV003247471 | pathogenic | Duchenne muscular dystrophy | 2022-03-01 | criteria provided, single submitter | clinical testing | Disruption of this splice site has been observed in individual(s) with Duchenne muscular dystrophy (PMID: 23536893). The frequency data for this variant in the population databases is considered unreliable, as metrics indicate poor data quality at this position in the gnomAD database. This sequence change affects a donor splice site in intron 26 of the DMD gene. RNA analysis indicates that disruption of this splice site induces altered splicing and may result in an absent or disrupted protein product. ClinVar contains an entry for this variant (Variation ID: 803890). For these reasons, this variant has been classified as Pathogenic. Studies have shown that disruption of this splice site results in activation of a cryptic splice site and introduces a premature termination codon (PMID: 23536893). The resulting mRNA is expected to undergo nonsense-mediated decay. |