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Behavioural Variant Frontotemporal Dementia (bvFTD)

Neural stem cells derived from Frontotemporal dementia (FTD) patient donor iPSCs and isogenic disease models 

Behavioural Variant Frontotemporal Dementia (bvFTD)

Frontotemporal dementia (FTD) refers to a spectrum of clinical diseases that may have several different underlying pathologies. Frontotemporal dementia can be sporadic or associated with mutations in the MAPT (microtubule-associated protein tau) and progranulin genes, and C9ORF72 repeat expansions.

These genetic mutations can lead to accumulation of TDP43 protein or tangles of MAPT (tau) protein. Currently, there are no approved treatments or reliable biomarkers for frontotemporal dementia. There is a need for in vitro human cell models for frontotemporal dementia and Alzheimer’s disease that can be used to accelerate research and assist in the development of new drugs to treat these disorders.

Behavioural Variant Frontotemporal Dementia (bvFTD)

Human iPSC-derived neural stem cells from Behavioural Variant Frontotemporal Dementia (bvFTD) patients

Fibroblasts from patients clinically diagnosed with Frontotemporal dementia were reprogrammed to iPSCs using our footprint-free episomal reprogramming method and then differentiated to neural stem cells using our chemically defined cortical neural induction method. Neural stem cells are available from FTD lines with mutations in MAPT genes, and from lines from Paget’s Disease patients, and patients exhibiting FTD and ALS symptoms.