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Abnormal Mitochondria

Mitochondria supply energy crucial to the survival of each and every cell. Abnormal, poorly functioning, or dying mitochondria are common in neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS). Unusual-appearing mitochondria appear early in animal models of ALS – even before disease symptoms surface.

Recent Packard research also shows that normal mitochondrial movement throughout motor neurons becomes erratic, although how this affects the availability of energy at key places in motor neurons, such as synapses, isn’t well understood. It’s a current topic of our Center’s grantees’ research.

Packard Center scientists have been actively pursuing possible biochemical causes of ailing, abnormal mitochondria in mouse and yeast models of ALS, in the hopes that understanding what’s happening to the tiny cell bodies will shed light on what starts the disease, both in familial and sporadic ALS.

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Johns Hopkins University
Motor neurons can only work properly if the cell’s proteins can get to the right place at the right time. Thomas Lloyd uses the fruit fly Drosophila melanogaster to study how proteins are shuttled between the cell body and the synapse, as interruptions in this process have been linked to ALS. 
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University of Michigan
Sami Barmada wants to answer a very basic question about ALS: why motor neurons? Of all the different types of neurons in the body (and scientists estimate there are probably several hundred), it’s only motor neurons that are affected in ALS. Knowing why this is, Barmada believes, could be the key to developing new potential treatments that could prevent the deterioration and death of motor neurons. 
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