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Lack of enzyme causes ALS says Italian study

Breakthrough spurs hope for new therapy of terminal illness

(ANSA) - ROME, JAN 5 - The lack of an enzyme in the body causes the fatal nerve-wasting disease Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig's Disease, an Italian study has shown, raising hope for new treatment options.
    The study by Milan's Mario Negri Institute and Turin's Città della Salute, published in the journal Brain, shows that the lack of cyclophillin A induces the terminal condition, which is also known as Motor Neuron(e) Disease (MND).
    Conducted in animal models and on humans, the breakthrough "could be a first important step towards developing a therapy," said the researchers.
    ALS is a neurodegenerative disease that results in the progressive loss of motor neurons that control voluntary muscles.
    The affected muscles are responsible for chewing food, speaking, and walking.
    Motor neuron loss continues until the ability to eat, speak, move, and finally the ability to breathe is lost.
    ALS eventually causes paralysis and early death, usually from respiratory failure.
    There is no known cure for ALS, and the goal of treatment is to improve symptoms.
    The disease became well known in the United States in the 20th century when in 1939 it affected baseball player Lou Gehrig and later worldwide following the 1963 diagnosis of cosmologist Stephen Hawking.
    The average survival from onset to death is two to four years, though this can vary, and about 10% survive longer than 10 years.
    In Europe, the disease affects about two to three people per 100,000 per year.
    It hits professional soccer players on average two times more than the general population and Serie A players are hit six times more, a study from Milan's Mario Negri Institute said in 2019.
    There have been several high-profile cases in Italy of right-to-die ALS sufferers committing suicide due to the hopelessness and physical incapacitation of the condition.


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