Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000182592 | SCV000234946 | pathogenic | not provided | 2017-01-17 | criteria provided, single submitter | clinical testing | This oathogenic variant is denoted c.2846delG at the cDNA level or at the protein level as p.Gly949GlufsX16. The normal sequence with the base that is deleted in braces is: AGGGG{G}AAAC. The c.2846delG variant in the RET gene causes a frameshift starting with codon Glycine 949, changes this amino acid to a Glutamic Acid residue and creates a premature Stop codon at position 16 of the new reading frame, denoted p.Gly949GlufsX16. This variant is predicted to cause loss of normal protein function either through protein truncation or nonsense-mediated mRNA decay. Although this variant has not been previously reported to our knowledge, its presence is consistent with a diagnosis of Hirschsprung disease. |