Mutations in CLP1, the first mammalian RNA kinase to be identified, could be contributing to the loss of motor neurons in motor neuron diseases such as ALS. In a surprising new study recently published in Nature, researcher discovered that mice carrying a mutant CLP1 gene had motor neuron loss leading to paralysis and sometimes death. The researchers found that when CLP1 was no longer functional, it increased the sensitivity of motor neurons to oxidative stress apparently through the accumulation of tRNA fragments. The CLP1-kinase inactive neurons responded to oxidative stress by activating p53, which caused the motor neurons to die. Removing p53 from mice with the CLP1 mutation prevented these motor neurons from dying. One of the researchers that worked on the study, Dr. Stefan Weitzer, said "We’ve discovered a new mechanism that leads to the death of motor neurons. If this holds true for other neuronal disease, our results could be one day used to drive the development of treatments for previously incurable diseases." Read more about these unexpected and interesting findings here.
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