Heterozygous mutations in the GRN, especially of the loss of function type, are causative of Frontotemporal dementia (FTD). However, several GRN variants can be found in other neurodegenerative diseases, such as Alzheimer’s disease (AD) and Parkinson’s disease. We reported 14 Chinese subjects with the rare variants of GRN (NM_002087.3). They presented with behavioral variant of FTD (n=2), primary progressive aphasia (n=4), AD (n=6), and mixed dementia (n=2). Six novel variants were found, including one likely pathogenic variant (p.W49X (c.146_147insA)) and five variants of uncertain significance (p.S226G (c.676A>G), p.M152I (c.456G>A), p.A91E (c.272C>A), p.G79E (c.236G>A), p.A303S (c. 907G>T)). These help us to better comprehend the genetic basis and therapeutic target of neurodegenerative diseases. (By Liling Dong, https://jmg.bmj.com/content/early/2024/01/16/jmg-2023-109499 )