Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV004699957 | SCV005205124 | pathogenic | Alstrom syndrome | 2024-06-06 | criteria provided, single submitter | clinical testing | Variant summary: ALMS1 c.5969C>G (p.Ser1990X), also known as c.5975C>G (S1991X) in the RefSeq database, results in a premature termination codon, predicted to cause a truncation of the encoded protein or absence of the protein due to nonsense mediated decay, which are commonly known mechanisms for disease. The variant was absent in 248890 control chromosomes (gnomAD). c.5969C>G has been reported in the literature in individuals affected with Alstrom Syndrome (e.g., Kilinc_2018, Bea-Mascato_2024). These data indicate that the variant is likely to be associated with disease. To our knowledge, no experimental evidence demonstrating an impact on protein function has been reported. No submitters have cited clinical-significance assessments for this variant to ClinVar. Based on the evidence outlined above, the variant was classified as pathogenic. |