Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001036394 | SCV001199755 | uncertain significance | Succinyl-CoA acetoacetate transferase deficiency | 2020-07-25 | criteria provided, single submitter | clinical testing | In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. Nucleotide substitutions within the consensus splice site are a relatively common cause of aberrant splicing (PMID: 17576681, 9536098). This variant has not been reported in the literature in individuals with OXCT1-related conditions. ClinVar contains an entry for this variant (Variation ID: 835501). This variant is present in population databases (rs534705079, ExAC 0.05%). This sequence change falls in intron 6 of the OXCT1 gene. It does not directly change the encoded amino acid sequence of the OXCT1 protein, but it affects a nucleotide within the consensus splice site of the intron. |
Prevention |
RCV003898042 | SCV004710643 | likely benign | OXCT1-related disorder | 2021-10-26 | no assertion criteria provided | clinical testing | This variant is classified as likely benign based on ACMG/AMP sequence variant interpretation guidelines (Richards et al. 2015 PMID: 25741868, with internal and published modifications). |