New findings about the development of motor neuron disease could help in the creation of treatments for the condition, British researchers say today.
Motor neuron disease, or amyotrophic lateral sclerosis (ALS), affects the function of nerve cells in the brain and spinal cord that control muscles.
A team from the Francis Crick Institute in London, UK, and University College London, UK, carried out tests on nerve cells from patients to examine how motor neuron disease begins and progresses at cellular and molecular levels.
They took skin cell samples from healthy volunteers and compared them to samples from patients with a genetic mutation that causes motor neuron disease. The cells from both groups were converted into a type of stem cell that can become many other cell types. Next, these cells were ‘guided’ into becoming motor neurons and astrocytes using specific chemical signals.
Dr Rickie Patani and the team found that astrocyte cells, which support neurons, may play a crucial role in the survival of motor neurons.
Dr Patani says: "Understanding how and why neurons die is clearly vital in neurodegenerative diseases, but part of the puzzle is also understanding the emerging role of astrocytes in this context.
"We were able to create pure, high-quality samples of motor neurons and astrocytes which accurately represent the cells affected in patients with motor neuron disease. Knowing when things go wrong inside a cell, and in what sequence, is a useful approach to define the ‘critical’ molecular event in disease."
The work was published yesterday (30 May) in Cell Reports.
The experts are now working with pharmaceutical companies on potential new treatments for motor neuron disease and other neurodegenerative diseases.
Patani, R. et al. Progressive motor neuron pathology and the role of astrocytes in a human stem cell model of VCP-related ALS. Cell Reports 30 May 2017; doi: 10.1016/j.celrep.2017.05.024 [abstract]
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