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Researchers "shocked" by Discovery that ALS-sick neurons show incredible resistance if given glucose



Increased glucose, transformed into energy, can give people with ALS improved mobility and longer life, according to a new study.

Doctors have long known that people with ALS are experiencing changes in their metabolism, which often lead to a rapid load loss in the ruthless cycle called hypermetabolism, according to a research team led by the Arizona University.

People with ALS use more energy while resting than those without the disease while at the same time they often struggle for effective glucose use, the exact ingredient the body needs to make more energy. Experts did not know exactly what was happening in the patient's cells to cause this dysfunction or how to relieve it.

"This project is a way of understanding these details," says lead author Ernesto Manzo, who describes the results published online at eLife as "really shocking."

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When ALS-affected neurons are given more glucose, they convert this source of energy into energy. With this energy they are able to survive longer and function better. Increasing glucose delivery in cells can be one way to respond to the unusually high energy needs of ALS patients. "These neurons find some relief by breaking down glucose and producing more cellular energy," Manso said. ALS is almost always a progressive disease, which ultimately takes away the patients' ability to walk, speak, and even breathe. The average life expectancy of the ALS patient from the time of diagnosis is two to five years.

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Previous studies on metabolism in ALS patients are focused on what happens on the whole body rather than on the cellular level, explained Daniela Zearnescu, Professor of Molecular and Cell Biology, and senior author of the study.

Researchers observed the brain lobes of the ALS-affected fruit flies under a microscope. The picture is a nervous cord, populated with green colored motor neurons expressing human glucose transporters. The areas of neurotransmitter release are shown in red and the muscles are shown in blue. (With the courtesy of the Zarnescu Laboratory)

"The fact that we found a compensation mechanism surprised me," Zserencu said. "These desperate, degenerating neurons show incredible resistance. This is an example of how unbelievable cells deal with stress. "

The news of the findings partly lies in the fact that the metabolism in ALS patients remains poorly understood, Zserscu said. partly because of limited access to the nervous system, "she said.

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Since scientists can not scavenge neurons from the brain without causing irreparable damage to a patient, researchers have used fruit flies as a model.

Fruit flies can teach us a lot about human diseases, "Manzo said.

In the lab, he and Zearnescu used powerful microscopes to observe the motor neurons of fruit flies in their larval state, paying great attention to what happened as they provided more glucose.

They found that when they increase the amount of glucose, motor neurons live longer and move more efficiently. When the researchers removed glucose from the neurons, the fruit fly larva moved slower. WATCH : The success of Parkinson's advanced stage is "beyond the wildest dreams of researchers"

with a pilot clinical experience that found a high carbohydrate diet is a possible intervention for patients with ALS with a severe metabolic dysfunction.

"Our data essentially explains why this approach can work," Zserencu said. "My goal is to convince clinicians to carry out a larger clinical trial to test this idea."

Reprinted from the University of Arizona

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