Mutations in the gene progranulin (PGRN) were recently identified as the cause of some
forms of frontotemporal dementia with ubiquitin-positive intraneuronal inclusion pathology
(FTLD-U). The DNA-binding protein, TDP-43, was determined to be a component of these
ubiquitinated inclusions in FTLD-U and amyotrophic lateral sclerosis (ALS) with dementia
(ALS-D). These findings raise many interesting questions as to the shared pathology and
possible common pathologic process between ALS and FTLD-U. This study examines the …