Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000480973 | SCV000570680 | likely pathogenic | not provided | 2016-06-13 | criteria provided, single submitter | clinical testing | The C231W variant in the FN1 gene has not been reported previously as a pathogenic variant, nor as a benign variant, to our knowledge. This variant was not observed in approximately 6500 individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, indicating it is not a common benign variant in these populations. The C231W variant is a non-conservative amino acid substitution, which is likely to impact secondary protein structure as these residues differ in polarity, charge, size and/or other properties. This substitution occurs at a position that is conserved across species, and in silico analysis predicts this variant is probably damaging to the protein structure/function. The C231W variant is a strong candidate for a pathogenic variant |
CHU Sainte- |
RCV000754912 | SCV000787522 | likely pathogenic | Spondylometaphyseal dysplasia | 2018-05-09 | no assertion criteria provided | research | 6 Individuals with novel FN1 mutations and spondylometaphyseal dysplasia |