Neuroprotective As A Compensatory Mechanism In ALS

ALS is a devastating disease; UA professor of molecular and cellular biology and senior author on the study. “It renders people from functioning one day to rapidly and visibly deteriorating; Previous studies on metabolism in ALS patients have focused primarily on what happens at the whole-body level; not the cellular level.

People with ALS use more energy while resting than those without the disease; while simultaneously they often struggle to effectively make use of glucose; the precise ingredient a body needs to make more energy. Experts have not known exactly what happens in a patient’s cells to cause this dysfunction or how to alleviate it. when ALS-affected neurons are given more glucose, they turn that power source into energy.

Molecular and cellular biology

But with that energy, they’re able to survive longer and function better. Increasing glucose delivery to the cells, then, may be one way to meet the abnormally high energy demands of ALS patients. These neurons were finding some relief by breaking down glucose and getting more cellular energy.

ALS is almost always a progressive disease, eventually taking away patients’ ability to walk, speak and even breathe. The average life expectancy of an ALS patient from the time of diagnosis is two to five years. These desperate, degenerating neurons showed incredible resilience. It is an example of how amazing cells are at dealing with stress. neurons from the brain without causing irreparable damage to a patient, the researchers used fruit flies as a model.

Metabolic dysfunction

By using used high powering microscopes to observe the motor neurons of fruit flies in their larval state; paying close attention to what happening as they providing more glucose. But finding that when they increasing the amount of glucose; the motor neurons lived longer and moved more efficiently.

When the researchers took glucose away from the neurons, the fruit fly larva moved more slowly. Their findings were consistent with a pilot clinical trial; which found a high carbohydrate diet was one possible intervention for ALS patients with gross metabolic dysfunction. Physicians have long known that people with ALS experience changes in their metabolism that often lead to rapid weight loss in a process called hypermetabolism.