Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV004818903 | SCV005438868 | uncertain significance | Ullrich congenital muscular dystrophy 1A | criteria provided, single submitter | clinical testing | The observed missense c.3502G>A p.Gly1168Arg variant in COL6A3 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Gly1168Arg variant is present with allele frequency of 0.0008% in gnomAD Exomes. This variant has not been submitted to the ClinVar database. Computational evidence Polyphen - Probably Damaging, SIFT - Tolerated and MutationTaster - Disease causing predicts conflicting evidence on protein structure and function for this variant. The reference amino acid of p.Gly1168Arg in COL6A3 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The amino acid Gly at position 1168 is changed to a Arg changing protein sequence and it might alter its composition and physico-chemical properties. For these reasons, this variant has been classified as a Variant of Uncertain Significance VUS. |