Patient-derived skin fibroblasts as a model to study frontotemporal lobar degeneration

Frontotemporal lobar degeneration(FTLD)is one of the most common causes of early-onset dementia in patients under the age of 65 years.It is a clinically,genetically,and neuropathologically heterogeneous group of neurodegenerative syndromes,causing atrophy in the temporal and frontal lobes of the brain.This is accompanied by progressive cognitive dysfunction,behavioral changes,difficulties in understanding or producing speech,and often also neuropsychiatric symptoms(Haapasalo and Remes,2015).Moreover,the clinical,genetic,and neuropathological features of FTLD may overlap with those of amyotrophic lateral sclerosis(ALS).Motor dysfunction is commonly present in FTLD patients and a portion of ALS patients experience frontal and temporal lobe dysfunction(Smith et al.,2019).