Total submissions: 1
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Laboratory for Molecular Medicine, |
RCV000155908 | SCV000205619 | likely pathogenic | Birt-Hogg-Dube syndrome | 2016-04-29 | criteria provided, single submitter | clinical testing | The p.Gly149fs variant in FLCN has been identified by our laboratory in 1 indivi dual with Birt-Hogg-Dube syndrome. It was absent from large population studies. This variant is predicted to cause a frameshift, which alters the protein?s amin o acid sequence beginning at position 149 and leads to a premature termination c odon 28 amino acids downstream. This alteration is then predicted to lead to a t runcated or absent protein. Frameshift and other truncating variants in FLCN ha ve been associated with Birt-Hogg-Dube syndrome and recurrent spontaneous pneumo thoraces (Nickerson 2002). In summary, although additional studies are required to fully establish its clinical significance, the p.Gly149fs variant is likely p athogenic. |