Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003856523 | SCV004697063 | likely benign | not provided | 2022-11-28 | criteria provided, single submitter | clinical testing | |
Neuberg Centre For Genomic Medicine, |
RCV004759329 | SCV005373635 | uncertain significance | Spinocerebellar ataxia type 5 | 2023-05-20 | criteria provided, single submitter | clinical testing | The missense c.6953C>T (p.Ser2318Leu) variant in the SPTBN2 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. This variant is reported with the allele frequency (0.001%) in the gnomAD Exomes. The amino acid Serine at position 2318 is changed to a Leucine changing protein sequence and it might alter its composition and physico-chemical properties. Computational evidence (Polyphen - Probably damaging, SIFT - Tolerated and MutationTaster - Disease causing) predicts conflicting evidence on protein structure and function for this variant. The amino acid change p.Ser2318Leu in SPTBN2 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Uncertain Significance. |