Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000760533 | SCV000890424 | pathogenic | not provided | 2018-01-31 | criteria provided, single submitter | clinical testing | The Q52X nonsense variant in the SLC16A2 gene is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The Q52X variant is not observed in large population cohorts (Lek et al., 2016). Although this pathogenic variant has not been reported previously to our knowledge, its presence is consistent with the diagnosis of an SLC16A2-related disorder in this individual. |
Duke University Health System Sequencing Clinic, |
RCV003223412 | SCV003918929 | pathogenic | Allan-Herndon-Dudley syndrome | 2023-04-20 | criteria provided, single submitter | research | |
Labcorp Genetics |
RCV003588676 | SCV004247271 | pathogenic | Spastic paraplegia | 2023-11-02 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Gln52*) in the SLC16A2 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in SLC16A2 are known to be pathogenic (PMID: 20083155, 25527620). This variant is not present in population databases (gnomAD no frequency). This variant has not been reported in the literature in individuals affected with SLC16A2-related conditions. ClinVar contains an entry for this variant (Variation ID: 620181). For these reasons, this variant has been classified as Pathogenic. |