Wed. Apr 17th, 2024
5-Aminolevulinic Acid's potential against mitochondrial disorders

Researchers from Tokyo Metropolitan University have made a groundbreaking discovery regarding the amino acid 5-aminolevulinic acid (5-ALA) and its potential to combat mitochondrial disorders. The study reveals that 5-ALA can aid in restoring deficiencies in Complex I (CI), the first of a series of protein complexes responsible for electron transportation and supporting mitochondrial energization. The findings offer new hope for developing effective treatments for these debilitating conditions.

Mitochondria, tiny organelles found in cells, play a crucial role in producing adenosine triphosphate (ATP), a chemical fuel that powers various biochemical processes in the human body. Mitochondrial disorders arise from issues with ATP production within the mitochondria. Particularly affected are energy-intensive organs like the brain and muscles, leading to severe conditions such as mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome.

Despite the severity of mitochondrial disorders, there is currently no cure. However, scientists are continuously exploring potential treatments. Complex I (CI) deficiency is a common cause of mitochondrial disorders. CI is responsible for transporting electrons to generate ATP. The research team, led by Associate Professor Kanae Ando, sought to find ways to counteract CI deficiency and boost ATP production using alternative protein complexes.

To test their hypothesis, the team conducted experiments on fruit flies with artificially reduced CI levels. These genetically engineered flies exhibited movement problems and shortened lifespans. However, when the flies were fed a combination of 5-aminovulenic acid and sodium ferrous citrate (SFC), their locomotor functions and neuromuscular junction development improved significantly. Surprisingly, the levels of ATP also increased, despite no direct elevation in CI levels.

Further analysis revealed that the activities of Complex II and IV, other protein complexes involved in electron transport and ATP production, were heightened by the supplementation of 5-ALA. The amino acid serves as a precursor to heme, a critical component in the function of Complex II, III, IV, and cytochrome c. Feeding the flies 5-ALA led to a metabolic shift that harnessed the power of Complex II and IV to transport electrons and produce ATP, effectively bypassing CI deficiency.

The researchers also noticed that the flies with CI deficiency had elevated levels of pyruvate and lactate in their cells, akin to human patients suffering from lactic acidosis due to CI deficiency. Remarkably, 5-ALA was found to reduce the levels of both substances in the flies, suggesting its potential therapeutic benefits.

Mitochondrial disorders pose complex challenges, and current treatments focus mainly on managing symptoms rather than addressing the root cause. The recent findings offer new insights and potential therapeutic options for combating these deadly diseases. As research progresses, the hope is that 5-aminolevulinic acid and its unique properties may hold the key to unlocking a more effective treatment approach, offering relief and improved quality of life for those affected by mitochondrial disorders.

By admin