Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000374180 | SCV000330756 | pathogenic | not provided | 2016-08-29 | criteria provided, single submitter | clinical testing | The c.4005+5_4005+8delGTAA variant in the KDM6A gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. This deletion changes the downstream intronic sequence including the conserved +5 position which results in the loss of the natural splice donor site in intron 27. This deletion is expected to cause abnormal gene splicing. The c.4005+5_4005+8delGTAA variant was not observed in approximately 6,500 individuals of European and African American ancestry in the NHLBI Exome Sequencing Project, indicating it is not a common benign variant in these populations. We interpret c.4005+5_4005+8delGTAA as a pathogenic variant. |