Mutations in the GRN gene coding for progranulin (PGRN) are responsible for many 
cases of familial frontotemporal lobar degeneration (FTLD) with TAR DNA-binding 
protein 43 (TDP-43)-positive inclusions (FTLD-TDP). GRN mutations create null 
alleles resulting in decreased progranulin protein or haploinsufficiency. 
FTLD-TDP with GRN mutations is characterized by lentiform neuronal intranuclear 
inclusions that are positive for TDP-43 in affected brain regions. In this 
study, by stably expressed short hairpin RNA, we established a neuroblastoma 
cell line with decreased PGRN level. This cell line reveals TDP-43-positive 
intranuclear inclusions. In addition, replacement with purified PGRN protein 
restores normal TDP-43 nuclear distribution. This cell model can be valuable for 
the study of the role of PGRN in the pathogenesis in FTLD-TDP.