Summary

Neurodegeneration refers to the progressive loss of neuron structure or function, which may eventually lead to cell death. Many neurodegenerative diseases, such as amyotrophic lateral sclerosis, multiple sclerosis, Parkinson's disease, Alzheimer's disease, Huntington's disease and prion disease, are the results of neurodegenerative processes. Neurodegeneration can be found in many different levels of neuronal circuits in the brain, from molecules to systems. Since there is no known method to reverse the progressive degeneration of neurons, these diseases are considered incurable. Biomedical research has revealed many similarities between these diseases at the subcellular level, including atypical protein assembly (such as protein diseases) and induction of cell death. These similarities indicate that progress in the treatment of one neurodegenerative disease may also improve other diseases. This collection of entries aims to collect various medical research results related to neurodegeneration. We invite researchers to share their new results and ideas related to neurodegeneration.

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Entries
Topic Review
Huntingtin and Other Neurodegeneration-Associated Proteins in Intracellular Pathologies
Neurodegenerative diseases are currently incurable. Numerous experimental data accumulated over the past fifty years have brought us closer to understanding the molecular and cell mechanisms responsible for their development. It is known that the basis of neurodegenerations are proteinopathies, disorders in the structure and function of various proteins that lead to their aggregation and toxic effects on cells. The most common neurodegenerative proteinopathies are amyloidosis (amyloid extracellular plaques in AD), tauopathy (various dementias), α-synucleopathy (Lowy bodies in PD), prionopathy, and TDP-43 proteinopathy (in amyotrophic lateral sclerosis (ALS)).
  • 594
  • 26 Dec 2022
Topic Review
Exercise-Boosted Mitochondrial Remodeling in Parkinson’s Disease
Parkinson’s disease (PD) is a movement disorder characterized by the progressive degeneration of dopaminergic neurons resulting in dopamine deficiency in the striatum. Given the estimated escalation in the number of people with PD, interventions aimed at minimizing morbidity and improving quality of life are crucial. Mitochondrial dysfunction and oxidative stress are intrinsic factors related to PD pathogenesis. Accumulating evidence suggests that patients with PD might benefit from various forms of exercise in diverse ways, from general health improvements to disease-specific effects and, potentially, disease-modifying effects.
  • 418
  • 23 Dec 2022
Topic Review
Aberrant Circadian Rhythm in Huntington’s Disease
Huntington’s disease (HD) is a progressive neurodegenerative disorder characterized by abnormal progressive involuntary movements, cognitive deficits, sleep disturbances, and psychiatric symptoms. Notably, gamma-aminobutyric acid (GABA)-ergic neurons that express the vasoactive intestinal peptide (VIP) in the brain play a key role in the regulation of circadian rhythm and neuroplasticity. While an abnormal clock gene pathway has been associated with the inactivation of GABAergic VIP neurons, recent studies suggest the activation of this neuronal population in the brain positively contributes to neuroplasticity.
  • 515
  • 22 Dec 2022
Topic Review
Lysophospholipids for Neurodegenerative Disorders
Neurodegenerative diseases (NDs) commonly present misfolded and aggregated proteins. Considerable research has been performed to unearth the molecular processes underpinning this pathological aggregation and develop therapeutic strategies targeting NDs. Fibrillary deposits of α-synuclein (α-Syn), a highly conserved and thermostable protein, are a critical feature in the development of NDs such as Alzheimer’s disease (AD), Lewy body disease (LBD), Parkinson’s disease (PD), and multiple system atrophy (MSA). Inhibition of α-Syn aggregation can thus serve as a potential approach for therapeutic intervention. The degradation of target proteins by small molecules has emerged as a new therapeutic modality, gaining the hotspot in pharmaceutical research. Additionally, interest is growing in the use of food-derived bioactive compounds as intervention agents against NDs via functional foods and dietary supplements. According to reports, dietary bioactive phospholipids may have cognition-enhancing and neuroprotective effects, owing to their abilities to influence cognition and mental health in vivo and in vitro. 
  • 393
  • 20 Dec 2022
Topic Review
Protein Glutathionylation and Glutaredoxin in Neurodegenerative Diseases
The brain is highly sensitive to oxidative stress due to its high oxygen consumption, abundance of unsaturated fatty acids which are prone to oxidation, and low antioxidant levels. It is a metabolically active and a high energy demanding organ that relies heavily on mitochondria for its energy needs. Majority of oxygen consumed by mitochondria during oxidative phosphorylation is coupled to ATP synthesis while ~4% contributes to the generation of superoxides which are further metabolized to reactive oxygen species (ROS). ROS modify proteins causing functional and structural damage to biomolecules. Prolonged exposure to reactive oxygen species (ROS) also damages DNA, mitochondrial membranes, and lipids, impairing its metabolic functions including synthesis of ATP, fatty acid oxidation and metabolism of essential biomolecules.
  • 838
  • 16 Dec 2022
Topic Review
Functional Foods to Ameliorate Neurogenic Muscle Atrophy
Neurogenic muscle atrophy is a debilitating condition that occurs from nerve trauma in association with diseases or during aging, leading to reduced interaction between motoneurons and skeletal fibers. Therapeutic approaches aiming at preserving muscle mass in a scenario of decreased nervous input include physical activity and employment of drugs that slow down the progression of the condition yet provide no concrete resolution. Nutritional support appears as a precious tool, adding to the success of personalized medicine, and could thus play a relevant part in mitigating neurogenic muscle atrophy.
  • 531
  • 15 Dec 2022
Topic Review
Glutathione Depletion and MicroRNA Dysregulation in MSA
Multiple system atrophy (MSA) is a rare neurodegenerative disease characterized by parkinsonism, cerebellar impairment, and autonomic failure. Although the causes of MSA onset and progression remain uncertain, its pathogenesis may involve oxidative stress via the generation of excess reactive oxygen species and/or destruction of the antioxidant system. One of the most powerful antioxidants is glutathione, which plays essential roles as an antioxidant enzyme cofactor, cysteine-storage molecule, major redox buffer, and neuromodulator, in addition to being a key antioxidant in the central nervous system. Glutathione levels are known to be reduced in neurodegenerative diseases. In addition, genes regulating redox states have been shown to be post-transcriptionally modified by microRNA (miRNA), one of the most important types of non-coding RNA. miRNAs have been reported to be dysregulated in several diseases, including MSA.
  • 416
  • 15 Dec 2022
Topic Review
Metabolic Dysfunction in ALS Skeletal Muscle
Defects in mitochondrial structure and function as well as energy metabolism in skeletal muscle contribute to disease pathology and progression, with metabolic dysfunctions appearing long before motor neuron degeneration and death.
  • 465
  • 15 Dec 2022
Topic Review
Extracellular Vesicles in Central Nervous System
Extracellular vesicles (EVs) form a heterogeneous group of membrane-enclosed structures secreted by all cell types. EVs export encapsulated materials composed of proteins, lipids, and nucleic acids, making them a key mediator in cell–cell communication. In the context of the neurovascular unit (NVU), a tightly interacting multicellular brain complex, EVs play a role in intercellular communication and in maintaining NVU functionality. In addition, NVU-derived EVs can also impact peripheral tissues by crossing the blood–brain barrier (BBB) to reach the blood stream. As such, EVs have been shown to be involved in the physiopathology of numerous neurological diseases.
  • 512
  • 13 Dec 2022
Topic Review
The Neuroprotective Potentiality of Flavonoids on Alzheimer’s Disease
Flavonoids are ubiquitous compounds of plants, produced by plants for growth and defense against all kinds of stress, including cold tolerance. More than 6000 different flavonoids have been identified, the primary sources of which are apples, red fruits, onions, citrus fruits, nuts, and beverages such as tea, coffee, beer, and red wine. These compounds, derived from phenol, are particularly interesting for their ability to cross the blood–brain barrier and for their multi-target activity. Several studies have described flavonoids to exhibit relevant biologic activities involving the neuronal antioxidants, as well as anti-amyloidogenic properties, acting as metal chelators, showing anti-inflammatory properties, and ameliorating cognition and neuroprotection.
  • 630
  • 12 Dec 2022
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