Advances in Mitochondrial Metabolism in Neurological Diseases

[Flyer]

Mitochondrial Function Assay

View

Mitochondria are important intracellular centers of energy metabolism, and their dysfunction is closely related to the pathogenesis of many neurological diseases. In recent years, studies on mitochondrial metabolism in neurological disorders have progressively deepened, revealing how mitochondrial dysfunction affects neuronal survival, function, and the health of the entire neural network.

Creative Biolabs describes recent research advances in mitochondrial metabolism in several major neurological disorders and offers the following related services to help accelerate the progress of your program.

Mitochondrial Metabolism in Alzheimer's Disease (AD)

AD is a common neurodegenerative disorder characterized by progressive cognitive decline and neuronal loss. Mitochondrial dysfunction has been found to be one of the important pathological features of AD.

• Changes in mitochondrial energy metabolism - Studies have shown that in neurons of AD patients, mitochondrial respiratory chain complex activity is significantly reduced and ATP synthesis is decreased.
  • Inadequate mitochondrial energy production leads to impaired neuronal energy metabolism, which in turn affects neuronal survival and function.
  • An imbalance in Ca²⁺ homeostasis within mitochondria is also thought to be closely related to the development of AD.
• Increased oxidative stress - Studies have shown significantly elevated levels of oxidative stress in the brain tissue of AD patients.
  • Excessive reactive oxygen species (ROS) damage the mitochondrial membrane and its DNA, causing further deterioration of mitochondrial function.
  • Mitochondrial damage is also thought to be one of the factors triggering the abnormal accumulation of β-amyloid, which further contributes to the progression of AD.
• Role of mitophagy - Recent studies have concluded that mitophagy plays an important role in AD. Mitochondrial autophagy maintains the healthy state of intracellular mitochondria by removing damaged mitochondria. Accordingly, dysfunction of mitochondrial autophagy is thought to be closely associated with AD-related neurodegenerative changes.

For mitochondrial metabolism related research, we also offer a range of research tools, including but not limited to the following:

Mitochondrial Metabolism in Parkinson's Disease (PD)

PD is a neurological disorder characterized by movement disorders in early childhood, which are mainly characterized by tremor, rigidity, and bradykinesia. Studies have shown that mitochondrial metabolic disorders are closely related to the development of PD.

• Mitochondrial dysfunction - Patients with PD have impaired mitochondrial function in dopaminergic neurons in the brain, as evidenced by decreased mitochondrial ATP synthesis and decreased activity of the respiratory chain complex. Mitochondrial dysfunction leads to insufficient energy production, resulting in decreased neuronal resistance to oxidative stress and ultimately cell death.
• Transcription factors and mitochondrial metabolism - The transcription factors PINK1 and Parkin also play an important role in the pathogenesis of PD. These factors are closely associated with mitochondrial autophagy and are involved in mitochondrial quality control.
• Mitochondria and the inflammatory response - Chronic inflammatory responses occur in the brain of PD patients, and recent studies have found that mitochondria release molecules that activate microglia and further exacerbate neuroinflammation.

Mitochondrial Metabolism in Huntington's Disease (HD)

HD is an inherited neurodegenerative disease characterized by the pathology of death of specific neurons, especially striatal neurons, leading to motor, emotional and cognitive dysfunction.

• Alterations in mitochondrial metabolism - Decreased mitochondrial membrane potential and dysregulation of mitochondrial dynamic homeostasis have been observed in both HD patients and animal models, leading to mitochondrial dysfunction. These abnormalities lead to decreased efficiency of oxidative phosphorylation, energy deficiency and increased apoptosis.
• SIRT1 and mitochondrial protection - The SIRT1 protein has been found to play a protective role in the pathological process of HD. SIRT1 can counteract the metabolic disturbances that occur in HD by promoting mitochondrial biogenesis and improved function through deacetylation.
• Mitochondria and neuroinflammation - Studies have also revealed that in HD, mitochondria-related signaling pathways regulate neuroinflammatory responses that influence disease progression.

Mitochondrial Metabolism in Multiple Sclerosis (MS)

MS is an autoimmune disease characterized by chronic inflammation and demyelination damage in the central nervous system. The role of mitochondrial metabolism in this disease has received equal attention.

Fig. 1 Mitochondrial dysfunction associated with multiple sclerosis.¹

• Disturbances in mitochondrial energy metabolism - Mitochondrial function is impaired in glial cells and neurons of MS patients, as evidenced by reduced efficiency of ATP production and oxidative phosphorylation. This metabolic impairment may accelerate neuronal cell death and myelin damage.
• Mitochondria and oxidative stress - Levels of oxidative stress are significantly elevated in the nervous system of MS patients, and mitochondria are one of the major sources of ROS production. Inhibition of mitochondria-generated ROS is thought to attenuate pathological damage in MS.

Abnormalities in mitochondrial metabolism in a variety of neurological disorders are closely linked to disease pathogenesis. With in-depth studies of mitochondrial function, mitochondria-based intervention strategies may be developed in the future to improve the course of these neurological diseases. By repairing mitochondrial function and improving cellular energy metabolism, it may have a positive impact on the treatment of neurological diseases.

Reference

1. Barcelos, Isabella Peixoto de, Regina M. Troxell, and Jennifer S. Graves. "Mitochondrial dysfunction and multiple sclerosis." Biology 8.2 (2019): 37, used under [CC BY 4.0](https://creativecommons.org/licenses/by/4.0/).

Related Scientific Resources

Neurodegenerative Disorders Drug Discovery Service