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
Relationship between D-Amino Acids and Schizophrenia
D-amino acids may play key roles for specific physiological functions in different organs including the brain. Importantly, D-amino acids have been detected in several neurological disorders such as schizophrenia, amyotrophic lateral sclerosis, and age-related disorders, reflecting the disease conditions. Relationships between D-amino acids and neurophysiology may involve the significant contribution of D-Serine or D-Aspartate to the synaptic function, including neurotransmission and synaptic plasticity. Gut-microbiota could play important roles in the brain-function, since bacteria in the gut provide a significant contribution to the host pool of D-amino acids. In addition, the alteration of the composition of the gut microbiota might lead to schizophrenia. Furthermore, D-amino acids are known as a physiologically active substance, constituting useful biomarkers of several brain disorders including schizophrenia.
  • 659
  • 17 May 2022
Topic Review
Human Induced Pluripotent Stem Cells-Based Models of AD
Alzheimer’s disease (AD) is an incurable neurodegenerative disorder and the leading cause of death among older individuals. Available treatment strategies only temporarily mitigate symptoms without modifying disease progression. Recent studies revealed the multifaceted neurobiology of AD and shifted the target of drug development. Established animal models of AD are mostly tailored to yield a subset of disease phenotypes, which do not recapitulate the complexity of sporadic late-onset AD, the most common form of the disease. The use of human induced pluripotent stem cells (HiPSCs) offers unique opportunities to fill these gaps. Emerging technology allows the development of disease models that recapitulate a brain-like microenvironment using patient-derived cells. These models retain the individual’s unraveled genetic background, yielding clinically relevant disease phenotypes and enabling cost-effective, high-throughput studies for drug discovery.
  • 369
  • 16 May 2022
Topic Review
Olfactory Evaluation in Model Mice
Olfactory dysfunction occurs in a variety of diseases, including COVID-19, neurodegenerative diseases, etc. This topic summarizes commonly used olfactory behavioral methods from the AD perspective.
  • 465
  • 16 May 2022
Topic Review
Post-Concussion Syndrome and Chronic Traumatic Encephalopathy
Traumatic brain injury is a significant public health issue and represents the main contributor to death and disability globally among all trauma-related injuries. Martial arts practitioners, military veterans, athletes, victims of physical abuse, and epileptic patients could be affected by the consequences of repetitive mild head injuries (RMHI) that do not resume only to short-termed traumatic brain injuries (TBI) effects but also to more complex and time-extended outcomes, such as post-concussive syndrome (PCS) and chronic traumatic encephalopathy (CTE). 
  • 581
  • 13 May 2022
Topic Review
Atypical Ubiquitination and Parkinson’s Disease
Ubiquitination (the covalent attachment of ubiquitin molecules to target proteins) is one of the main post-translational modifications of proteins. Historically, the type of polyubiquitination, which involves K48 lysine residues of the monomeric ubiquitin, was the first studied type of ubiquitination. It usually targets proteins for their subsequent proteasomal degradation. All the other types of ubiquitination, including monoubiquitination; multi-monoubiquitination; and polyubiquitination involving lysine residues K6, K11, K27, K29, K33, and K63 and N-terminal methionine, were defined as atypical ubiquitination (AU). Good evidence now exists that AUs, participating in the regulation of various cellular processes, are crucial for the development of Parkinson’s disease (PD). These AUs target various proteins involved in PD pathogenesis. The K6-, K27-, K29-, and K33-linked polyubiquitination of alpha-synuclein, the main component of Lewy bodies, and DJ-1 (another PD-associated protein) is involved in the formation of insoluble aggregates. Multifunctional protein kinase LRRK2 essential for PD is subjected to K63- and K27-linked ubiquitination. Mitophagy mediated by the ubiquitin ligase parkin is accompanied by K63-linked autoubiquitination of parkin itself and monoubiquitination and polyubiquitination of mitochondrial proteins with the formation of both classical K48-linked ubiquitin chains and atypical K6-, K11-, K27-, and K63-linked polyubiquitin chains. The ubiquitin-specific proteases USP30, USP33, USP8, and USP15, removing predominantly K6-, K11-, and K63-linked ubiquitin conjugates, antagonize parkin-mediated mitophagy. 
  • 406
  • 13 May 2022
Topic Review
Omics Sciences and Amyotrophic Lateral Sclerosis
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease caused by the progressive loss of motor neurons (MNs) resulting in weakness and paralysis of voluntary muscles. The main clinical feature of ALS is the upper and lower MN involvement. The age of onset is about 60 years, and the incidence is 5 per 100,000 inhabitants. Although important research progress has been made, the etiopathology of ALS is mostly unknown. The mechanisms underlying the development of the disease are multiple, with the involvement of a complex interaction between genetic and molecular characteristics. The major ALS-related genes include superoxide dismutase 1 (SOD1), FUSed in sarcoma (FUS), TAR DNA binding protein (TARDBP) and chromosome 9 open reading frame 72 (C9Orf72). Due to the high complexity of the disease, the diagnosis is made by exclusion and there are no effective drug therapies that can stop or significantly slow down the progression of the disease. To date, the drugs used to slow down the course of the disease are Riluzole, which works by reducing excitotoxicity, and Edaravone that decreases oxidative stress. Neuropathology and medical genetics have led to the discovery that ALS and Frontotemporal Dementia (FTD) are related diseases and form a broad neurodegenerative continuum. Both of these pathologies can be caused by mutations in the same gene that can lead to different clinical pictures. The discovery of hexanucleotide expansion involving the C9Orf72 gene helped to define a genetic basis to explain the spectrum ALS/FTD.
  • 476
  • 13 May 2022
Topic Review
Autosomal Dominant Lateral Temporal Epilepsy
Autosomal dominant lateral temporal epilepsy (ADLTE) is a genetic focal epilepsy associated with mutations in the LGI1, RELN, and MICAL1 genes. A previous study linking ADLTE with two MICAL1 mutations that resulted in the substitution of a highly conserved glycine residue for serine (G150S) or a frameshift mutation that swapped the last three C-terminal amino acids for 59 extra residues (A1065fs) concluded that the mutations increased enzymatic activity and promoted cell contraction.
  • 706
  • 11 May 2022
Topic Review
Glutamatergic System in Depression
Major depressive disorder (MDD) is a heterogeneous neuropsychological disorder characterized by a combination of symptoms that negatively impact the productivity and well-being of inflicted patients, including impairments in cognition, emotional regulation, memory, motor function, motivation, and possible suicidal ideation. Glutamate is the major excitatory neurotransmitter in the central nervous system. It plays an important role in several physiological functions.
  • 2.7K
  • 11 May 2022
Topic Review
Mechanism of Action of Carnosine
Carnosine is a natural endogenous molecule that has been extensively studied during the last years due to its promising beneficial effects for human health. It presents multimodal mechanisms of action, being able to exert antioxidant, anti-inflammatory and anti-aggregate activities, among others.
  • 1.3K
  • 11 May 2022
Topic Review
Antioxidant Effects on the Brain and Skeletal Muscle
Erythroid-related nuclear factor 2 (NRF2) and the antioxidant-responsive-elements (ARE) signaling pathway are the master regulators of cell antioxidant defenses, playing a key role in maintaining cellular homeostasis, a scenario in which proper mitochondrial function is essential. Increasing evidence indicates that the regular practice of physical exercise increases cellular antioxidant defenses by activating NRF2 signaling.
  • 404
  • 10 May 2022
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