Mitochondria ALS, or Amyotrophic Lateral Sclerosis, is an evolving area of research that has been gaining increasing attention. This non-cellular organelle, commonly known as the powerhouse of the cell, can be implicated in the pathogenesis of ALS. ALS is a degenerative disease implicating motor neurons in the brain and spinal cord, with patients usually passing away from respiratory failure within 2-5 years after symptoms first appear. Our understanding of mitochondrial dysfunction in ALS, along with its link to energy failure in neurons, is shedding new light on the emerging treatment approaches.
Understanding Mitochondria in ALS
In order to elucidate the role of mitochondria in ALS, we need to understand its functions. The main function of mitochondria is to produce adenosine triphosphate (ATP), the energy that our cells use for survival. But beyond powering our cells, they also regulate cell cycle, cell growth, and even programmed cell death (1).
In the case of ALS, one of the most accepted theories posits that the motor neurons die due to a process referred to as oxidative stress. Studies demonstrate the distinct mitochondrial abnormalities in ALS; similar work has also focused on how and why these organelles become disrupted in ALS (2).
Mitochondria ALS and Oxidative Stress
Definitionally, oxidative stress is an imbalance between the generation of reactive oxygen species (ROS) and the ability to detoxify these reactive products or repair the resulting damage. In a regular environment, there’s a natural balance of ROS production and detoxification. However, imbalance in favor of ROS leads to oxidative stress.
In the ALS context, both human and animal model studies have concluded this oxidative stress and mitochondrial damage as a pathological hallmark of the disease (3).
Energy Failure Neurons: The Link with Mitochondrial ALS
Further research has brought attention to the perspective of energy failure neurons as significant contributors to ALS pathology. As neurons are highly dependent upon oxidative metabolism, neuronal energy failure seems a plausible consequence of mitochondrial dysfunction. Several studies indicate that there indeed exists metabolic dysregulation within the motor neurons’ mitochondria in ALS (4).
This energy failure in neurons can lead to various detrimental effects such as inability to send signals, maintain resting potential, restore ionic gradients after action potentials, or removal of synaptic neurotransmitters, ultimately leading to neuronal death.
New Insights into Possible Treatment Approaches
Several novel ALS treatments are now in clinical trials, some of which are targeting energy metabolism. For example, Tolar’s drug CNM-Au8 is a suspension of gold nanocrystals that scientists believe improves neuronal survival by enhancing functions of neuron mitochondria (5).
Excellent progress is being made in our understanding of how ALS affects energy production in neurons. Nevertheless, it is important to remember that ALS is a highly complex disease, likely involving many different factors. However, the mitochondrial theory of ALS offers hope and a new path forward to slow disease progression and potentially find a cure.
Getting Support and Assistance
Mitochondria ALS and the energy failure in neurons are more complicated than what a single article can capture. Thereby, for those facing ALS or who think Real Water may have contributed to their condition, seeking reliable resources and institutional support becomes crucial.
I invite my readers to explore further about ALS, its intricate relationships with oxidative stress, energy failure neurons, and the groundbreaking therapeutic developments on our blog.
If you or your loved ones are living with ALS, remember you are not alone. Reach out to us through the contact page on our ALS Real Water Las Vegas website or call 702-385-6000 for immediate assistance. Together, we can better understand, manage and eventually, conquer this disease.
References
1. “The Role of Mitochondria in ALS“, Biochimica et Biophysica Acta (BBA)—Molecular Basis of Disease
2. “Mitochondrial dysfunction in ALS30288-X)”, The Lancet Neurology
3. “Interplay of oxidative stress and neuroinflammation in ALS“, Cellular and Molecular Life Sciences
4. “Mitochondrial metabolism and the control of vascular smooth muscle cell proliferation“, Frontiers in Cell and Developmental biology
5. “Gold drug for ALS is ready to go to clinical trial“, ALS Therapy Development Institute