Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV000895771 | SCV001039836 | benign | not provided | 2022-11-15 | criteria provided, single submitter | clinical testing | |
Revvity Omics, |
RCV000895771 | SCV003809227 | uncertain significance | not provided | 2020-09-29 | criteria provided, single submitter | clinical testing | |
Neuberg Centre For Genomic Medicine, |
RCV003338846 | SCV004048473 | uncertain significance | Schwartz-Jampel syndrome type 1 | criteria provided, single submitter | clinical testing | The missense variant c.10028G>A (p.Arg3343Lys) in HSPG2 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The p.Arg3343Lys variant is novel (not in any individuals) in 1000 Genomes and allele frequency of 0.04061% is reported in gnomAD. The amino acid Arg at position 3343 is changed to a Lys changing protein sequence and it might alter its composition and physico-chemical properties. In silico tools predict the variant to be tolerated. The residue is conserved across species. The amino acid change p.Arg3343Lys in HSPG2 is predicted as conserved by GERP++ and PhyloP across 100 vertebrates. For these reasons, this variant has been classified as Uncertain Significance. |