Motor Neuron Disease and Frontotemporal Dementia Consortium

Motor neurons derived from induced pluripotent stem cells. The stem cells were generated using skin tissue from a patient with a mutation in a protein called TDP-43 (M337V mutation).
Green: Choline acetyl transferase, marker of motor neurons; Red: TDP-43, the mutant protein; Blue: cell nuclei. Contributed by Dr. Agnes Nishimura, King’s College London

RNA processing proteins in neurodegeneration

Mutations in proteins called TDP-43 and FUS are linked to the development of motor neuron disease. These proteins share common functions- both act to transport and splice (cut and rejoin) RNA, the strips of information sent from the DNA to instruct cells to produce other proteins. The Motor Neuron Disease and Frontotemporal Dementia Consortium is focused on the role of RNA processing proteins such as TDP-43 and FUS in brain degeneration.  The consortium is headed by Professor Christopher Shaw at King’s College London, and comprises several groups at King’s College London (Dr. Jean-Marc Gallo, Prof. Noel Buckley, Prof. Corinne Houart), as well as groups at the University of California (Prof. Don Cleveland), University of Cambridge (Dr. Jerne Ule), University of Dundee (Prof. John Rouse), and University of Manchester (Profs. Stuart Pickering-Brown and David Mann). The consortium uses a variety of approaches to investigate whether brain degeneration is related to loss of the normal function of these RNA processing proteins, or gain of a new, toxic function. The overarching aim of the consortium is to help to identify therapies for the treatment of motor neuron disease and and frontotemporal dementia.

For more information about our consortium, please see The Role of RNA-Processing Proteins in Neurodegeneration website.


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