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Scientists at the University of Sheffield have successfully created a zebrafish carrying the complex genetic change known to be at the origin of the most common genetic form of motor neuron disease (MND).
This breakthrough will accelerate innovative research and experimental trials of drugs to fight degenerative disease.
Until now, the research aimed to better understand how the disease presented itself and experimental drug trials were conducted on models of fruit flies or mice. This has had limited success due to the difference between the human brain and the fruit fly brain and implications in terms of time and costs of using mouse models.
For the first time, researchers at the Institute of Translational Neuroscience (SITraN) at the University of Sheffield have successfully created the complex aspects of human pathobiology C9-ALS / FTD in zebrafish models .
This pioneering breakthrough is essential for studying the underlying mechanisms of MND and Frontotemporal Dementia (FTD).
MND, also known as amyotrophic lateral sclerosis (ALS), is a devastating neurodegenerative disease that affects the nerves, motor neurons, brain and spinal cord, which tell the muscles what to do.
The messages of these nerves gradually stop reaching the muscles, weakening them, stiffening them and eventually wasting them. Progressive disease affects the patient's ability to walk, talk, eat and breathe. MND affects 5,000 adults in the UK and there is currently no cure.
About 10% of cases of MND are hereditary, but the remaining 90% are due to complex genetic and environmental interactions that are not well understood at the present time – that's what called sporadic MND.
The most common genetic cause known from MND and FTD is an expansion of hexanucleotide in the first intron of the C9orf72 gene. This gene contains hundreds and thousands of repeats of the GGGGCC sequence in patients with MND. This mutation is the largest genetic cause of MND and also the most predominant form of sporadic ALS.
Dr. Tennore Ramesh, of SITRAN of the University of Sheffield, said: "The use of zebrafish models for DMN research means that we can accelerate studies and our understanding of the disease. devastating and other neurological conditions.
"Because zebrafish are transparent, you can record study results much more quickly and easily – research is much less invasive.
"Trying 1,000 drugs on mouse models would take more than 10 years, but testing 1,000 drugs on zebrafish would only take a few months.
"This will allow us to accelerate research on clinical trials in humans faster than ever before."
The four-year project, led by Dr. Ramesh in collaboration with leading researchers from SITRAN, including Professor Dame Pamela Shaw, Vice President and Director of the Faculty of Medicine, Dentistry and Health of the University of Sheffield, is published in the newspaper Acta Neuropathologica Communications. The study was funded by the MND association.
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More information:
Matthew P. Shaw et al., Stable Transgenic Zebrafish Model C9orf72, Key Aspects of the ALS / FTD Phenotype and Revealing New Pathological Features, Acta Neuropathologica Communications (2018). DOI: 10.1186 / s40478-018-0629-7
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