Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Counsyl | RCV000410476 | SCV000485758 | likely pathogenic | Cohen syndrome | 2016-02-09 | criteria provided, single submitter | clinical testing | |
Labcorp Genetics |
RCV000410476 | SCV004476354 | pathogenic | Cohen syndrome | 2023-01-20 | criteria provided, single submitter | clinical testing | For these reasons, this variant has been classified as Pathogenic. ClinVar contains an entry for this variant (Variation ID: 370433). This premature translational stop signal has been observed in individual(s) with Cohen syndrome (PMID: 12730828). This variant is not present in population databases (gnomAD no frequency). This sequence change creates a premature translational stop signal (p.Trp2824*) in the VPS13B gene. It is expected to result in an absent or disrupted protein product. Loss-of-function variants in VPS13B are known to be pathogenic (PMID: 15141358, 16648375, 20461111). |