Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000290744 | SCV000330594 | pathogenic | not provided | 2016-06-14 | criteria provided, single submitter | clinical testing | To our knowledge, the c.574dupA pathogenic variant in the ALDH3A2 gene has not been reported previously as a pathogenic variant nor as a benign variant. It causes a frameshift starting with codon Isoleucine 192, changes this amino acid to an Asparagine residue, and creates a premature Stop codon at position 16 of the new reading frame, denoted p.p.Ile192AsnfsX16. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. In addition, the c.574dupA 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.574dupA as a pathogenic variant. |
Counsyl | RCV000409660 | SCV000486164 | likely pathogenic | Sjögren-Larsson syndrome | 2016-04-08 | criteria provided, single submitter | clinical testing |