Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000484582 | SCV000573555 | uncertain significance | not provided | 2017-03-01 | criteria provided, single submitter | clinical testing | The c.1009delA variant in the GABRA6 gene has not been reported previously as a pathogenic variant nor as a benign variant, to our knowledge. The c.1009delA variant causes a frameshift starting with codon Arginine 337, changes this amino acid to a Glycine residue, and creates a premature Stop codon at position 11 of the new reading frame, denoted p.Arg337GlyfsX11. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. The c.1009delA variant is not observed at a significant frequency in large population cohorts (Lek et al., 2016; 1000 Genomes Consortium et al., 2015; Exome Variant Server). We interpret c.1009delA as a variant of uncertain significance. |
Genome |
RCV000509493 | SCV000607041 | not provided | GABRA6-related disorder | no assertion provided | phenotyping only | GenomeConnect assertions are reported exactly as they appear on the patient-provided report from the testing laboratory. GenomeConnect staff make no attempt to reinterpret the clinical significance of the variant. |