Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV004785910 | SCV005401033 | uncertain significance | Pontocerebellar hypoplasia, type 14 | criteria provided, single submitter | clinical testing | The observed missense c.344C>A (p.Thr115Asn) variant in PPIL1 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Thr115Asn variant is absent in gnomAD Exomes. This variant has not been submitted to the ClinVar database. Multiple lines of computational evidence (Polyphen - Probably damaging, SIFT – Damaging and Mutation Taster - Disease causing) predict a damaging effect on protein structure and function for this variant. The reference amino acid at this position on PPIL1 gene is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. The amino acid Thr at position 115 is changed to a Asn changing protein sequence and it might alter its composition and physico-chemical properties. For these reasons, this variant has been classified as Variant of Uncertain Significance (VUS). |