It was recently shown, that inheritance of pathogenic STUB1 variants in combination with TBP alleles of reduced penetrance is frequently of digenic-caused disease with mutant CHIP and TBP has not been definitively elucidated, STUB1 and TBP should be included in diagnostic genetic testing panels for patients with early onset dementia syndromes that resemble FTD. The gene discussed is STUB1; the disease is frontotemporal dementia.