‘Self-eating’ mitochondria may make the brain vulnerable to Lou Gehrig’s disease

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Stephen Hawking

A newly discovered type of mitochondrial self-destruction may make some brain cells vulnerable to ALS, also known as Lou Gehrig’s disease.

In mice genetically engineered to develop some forms of a degenerative nerve disease similar to amyotrophic lateral sclerosis, energy-generating organelles called mitochondria appear to dismantle themselves without help from usual cell demolition crews.

This type of power plant self-destruction was spotted in upper motor neurons, brain nerve cells that help initiate and control movements, but not in neighboring cells, researchers report November 7 in Frontiers in Cellular Neuroscience. Death of those upper motor neurons is a hallmark of ALS, and the self-destructing mitochondria may be an early step that sets those cells up to die later.

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Pembe Hande Özdinler, a cellular neuroscientist at Northwestern University Feinberg School of Medicine in Chicago, and her colleagues have dubbed the mitochondrial dissolution “mitoautophagy.”

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“It’s self-eating itself. That’s why we said, ‘This isn’t normal. This we have never seen before,’” Özdinler says. Self-eating mitochondria may somehow make upper motor neurons more vulnerable to ALS later in life. Details of that vulnerability haven’t been worked out yet.

Read full, original post: Self-destructing mitochondria may leave some brain cells vulnerable to ALS

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