Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Women's Health and Genetics/Laboratory Corporation of America, |
RCV001806794 | SCV002051237 | likely pathogenic | Al-Raqad syndrome | 2021-12-18 | criteria provided, single submitter | clinical testing | Variant summary: DCPS c.454C>T (p.Arg152X) 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. Truncations downstream of this position have not been classified as pathogenic by our laboratory nor reported in the HGMD/LOVD databases. The variant allele was found at a frequency of 4e-06 in 251474 control chromosomes. To our knowledge, no occurrence of c.454C>T in individuals affected with Al-Raqad Syndrome and no experimental evidence demonstrating its impact on protein function have been reported. No clinical diagnostic laboratories have submitted clinical-significance assessments for this variant to ClinVar after 2014. Based on the evidence outlined above, the variant was classified as likely pathogenic. |