Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Baylor Genetics | RCV000735813 | SCV004212938 | likely pathogenic | Nephropathic cystinosis | 2023-10-12 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV003768260 | SCV004586944 | pathogenic | Ocular cystinosis; Juvenile nephropathic cystinosis; Inborn genetic diseases | 2023-01-27 | criteria provided, single submitter | clinical testing | This variant is not present in population databases (gnomAD no frequency). For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 599228). This variant has not been reported in the literature in individuals affected with CTNS-related conditions. This sequence change creates a premature translational stop signal (p.Val257Serfs*39) in the CTNS gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in CTNS are known to be pathogenic (PMID: 9537412, 27102039). |
Institute of Human Genetics, |
RCV000735813 | SCV000863546 | likely pathogenic | Nephropathic cystinosis | no assertion criteria provided | clinical testing |