Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Lupski Lab, |
RCV004696611 | SCV005187361 | pathogenic | Hypercholanemia, familial | 2024-08-13 | criteria provided, single submitter | clinical testing | This variant was identified as homozygous in one Turkish individual with neurodevelopmental delay. The variant is absent in gnomAD. In vivo studies in zebrafish suggest wdr83os loss of function is responsible for the human phenotype observed. Other variants in this gene were identified in association with neurodevelopmental delay and hypercholanemia, although bile salt measurements were not available for this patient/variant. |
OMIM | RCV004794694 | SCV005415395 | pathogenic | Neurodevelopmental disorder with variable familial hypercholanemia | 2024-11-25 | no assertion criteria provided | literature only |