Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV003086852 | SCV003483012 | uncertain significance | Cohen syndrome | 2021-12-22 | criteria provided, single submitter | clinical testing | This variant, c.11647_11649del, results in the deletion of 1 amino acid(s) of the VPS13B protein (p.Glu3883del), but otherwise preserves the integrity of the reading frame. This variant is present in population databases (no rsID available, gnomAD 0.007%). This variant has not been reported in the literature in individuals affected with VPS13B-related conditions. Experimental studies and prediction algorithms are not available or were not evaluated, and the functional significance of this variant is currently unknown. In summary, the available evidence is currently insufficient to determine the role of this variant in disease. Therefore, it has been classified as a Variant of Uncertain Significance. |
Prevention |
RCV004744584 | SCV005366210 | uncertain significance | VPS13B-related disorder | 2024-09-04 | no assertion criteria provided | clinical testing | The VPS13B c.11572_11574delGAG variant is predicted to result in an in-frame deletion (p.Glu3858del). To our knowledge, this variant has not been reported in the literature. This variant is reported in 0.0065% of alleles in individuals of European (Non-Finnish) descent in gnomAD. At this time, the clinical significance of this variant is uncertain due to the absence of conclusive functional and genetic evidence. |