Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Neuberg Centre For Genomic Medicine, |
RCV004577305 | SCV005061268 | likely pathogenic | Nephropathic cystinosis | criteria provided, single submitter | clinical testing | The splice donor c.140+2dup variant in the CTNS 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 (0.0003%) in the gnomAD Exomes and novel (not in any individuals) in 1000 Genomes. This variant change affects the donor splice site in intron 4 of the CTNS gene. Loss of function variants has been previously reported to be disease causing (Brasell et al., 2019). For these reasons, this variant has been classified as Likely Pathogenic. |