Total submissions: 3
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001205716 | SCV001376986 | pathogenic | Nephronophthisis | 2024-10-29 | criteria provided, single submitter | clinical testing | This sequence change creates a premature translational stop signal (p.Arg951*) in the NPHP3 gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in NPHP3 are known to be pathogenic (PMID: 18371931, 23559409). This variant is present in population databases (rs148670389, gnomAD 0.006%). This premature translational stop signal has been observed in individual(s) with NPHP3-related conditions (internal data). ClinVar contains an entry for this variant (Variation ID: 936828). For these reasons, this variant has been classified as Pathogenic. |
Fulgent Genetics, |
RCV002504239 | SCV002807969 | likely pathogenic | Renal-hepatic-pancreatic dysplasia 1; Nephronophthisis 3; NPHP3-related Meckel-like syndrome | 2024-04-20 | criteria provided, single submitter | clinical testing | |
Prevention |
RCV004528417 | SCV004106016 | likely pathogenic | NPHP3-related disorder | 2024-01-05 | no assertion criteria provided | clinical testing | The NPHP3 c.2851C>T variant is predicted to result in premature protein termination (p.Arg951*). To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.0065% of alleles in individuals of South Asian descent in gnomAD. Nonsense variants in NPHP3 are expected to be pathogenic. This variant is interpreted as likely pathogenic. |