23.08.2023 | Published by bit.bio

Disease model panel to advance Frontotemporal dementia drug discovery

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bit.bio has launched ioGlutamatergic Neurons MAPT N279K/N279K - completing a panel of ioDisease Model Cells for studying Frontotemporal dementia (FTD) and tauopathies. 

The disease models were co-developed with our partners Charles River Laboratories, who characterised the cells and used them to develop automated high-content imaging assays. The Charles River team observed hyperphosphorylation of tau in the disease model cells compared to the wild-type genetically matched control. Hyperphosphorylation of tau is a pathological hallmark of tauopathies, such as FTD and Alzhiemer’s disease (AD).  The data supports the use of these disease model cells as a physiologically relevant model to investigate the impact of mutant tau protein on disease progression.

ioGlutamatergic Neurons MAPT N279K/N279K are opti-ox precision reprogrammed glutamatergic neurons carrying a genetically engineered homozygous N279K mutation in the MAPT gene encoding the tau protein. The product completes the panel of four neuronal disease models for FTD that includes a heterozygous MAPT N279K mutation, a homozygous MAPT P301S mutation and a heterozygous MAPT P301S mutation.  

The genetic mutations N279K and P301S in the MAPT gene cause the production of abnormal tau protein, which aggregates and forms neurofibrillary tangles that accumulate and disrupt normal cellular functions. This results in the degeneration of nerve cells in the frontal and temporal lobes of the brain and leads to changes in personality, behaviour and language.

"FTD is a progressive, fatal neurodegenerative disease and is the second leading cause of early onset dementia following Alzheimer’s disease. There are currently no treatments available to stop it or slow it down. Our panel of products can be used to investigate these diseases at the molecular and cellular level, are a relevant model to bridge the gap between animal and human studies, and have the potential to aid the development of therapeutics."




    Amanda Turner
    bit.bio Senior Product Manager



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