Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Fulgent Genetics, |
RCV005035039 | SCV005664943 | pathogenic | Hermansky-Pudlak syndrome 3 | 2024-04-07 | criteria provided, single submitter | clinical testing | |
Gene |
RCV005230823 | SCV005874646 | pathogenic | not provided | 2024-08-27 | criteria provided, single submitter | clinical testing | Observed with a second HPS3 variant in unrelated patients with Hermansky-Pudlak syndrome in the published literature, but it is not known whether the variants occurred on the same (in cis) or on different (in trans) chromosomes in all cases (PMID: 27593200, 32725903); Nonsense variant predicted to result in protein truncation or nonsense mediated decay in a gene for which loss of function is a known mechanism of disease; Not observed at significant frequency in large population cohorts (gnomAD); This variant is associated with the following publications: (PMID: 31898847, 32725903, 27593200) |