Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Gene |
RCV000200843 | SCV000251458 | pathogenic | not provided | 2013-09-05 | criteria provided, single submitter | clinical testing | The c.1349_1352delATGA mutation in the FH gene causes a frameshift starting with codon Asparagine 450, changes this amino acid to a Serine residue, and creates a premature Stop codon at position 3 of the new reading frame, denoted p.Ans450SerfsX3. This mutation is predicted to cause loss of normal protein function through protein truncation. Although this mutation has not been previously reported to our knowledge, its presence is consistent with a diagnosis of HLRCC. The variant is found in FH panel(s). |