Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000542955 | SCV000650580 | benign | Aortic valve disease 2 | 2024-01-19 | criteria provided, single submitter | clinical testing | |
Women's Health and Genetics/Laboratory Corporation of America, |
RCV004701636 | SCV005205333 | likely benign | not specified | 2024-06-04 | criteria provided, single submitter | clinical testing | Variant summary: SMAD6 c.139C>T (p.Pro47Ser) results in a non-conservative amino acid change in the encoded protein sequence. Three of five in-silico tools predict a benign effect of the variant on protein function. The variant allele was found at a frequency of 0.00081 in 33256 control chromosomes. The observed variant frequency is approximately 26-fold of the estimated maximal expected allele frequency for a pathogenic variant in SMAD6 causing Aortic Valve Disease phenotype (3.1e-05). To our knowledge, no occurrence of c.139C>T in individuals affected with Aortic Valve Disease and no experimental evidence demonstrating its impact on protein function have been reported. ClinVar contains an entry for this variant (Variation ID: 471749). Based on the evidence outlined above, the variant was classified as likely benign. |