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
The Primary Microglial Leukodystrophies
Primary microglial leukodystrophy or leukoencephalopathy are disorders in which a genetic defect linked to microglia causes cerebral white matter damage. Pigmented orthochromatic leukodystrophy, adult-onset orthochromatic leukodystrophy associated with pigmented macrophages, hereditary diffuse leukoencephalopathy with (axonal) spheroids, and adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP) are different terms apparently used to designate the same disease. However, ALSP linked to dominantly inherited mutations in CSF1R (colony-stimulating factor receptor 1) causes CSF-1R-related leukoencephalopathy (CRP). Yet, recessive ALSP with ovarian failure linked to AARS2 (alanyl-transfer (t)RNA synthase 2) mutations (LKENP) is a mitochondrial disease and not a primary microglial leukoencephalopathy. Polycystic membranous lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL; Nasu–Hakola disease: NHD) is a systemic disease affecting bones, cerebral white matter, selected grey nuclei, and adipose tissue The disease is caused by mutations of one of the two genes TYROBP or TREM2, identified as PLOSL1 and PLOSL2, respectively.
  • 529
  • 30 Jun 2022
Topic Review
The Dementia with Lewy Bodies
Dementia with Lewy Bodies (DLB) is a common form of cognitive neurodegenerative disease. Only one third of patients are correctly diagnosed due to the clinical similarity mainly with Alzheimer’s disease (AD).
  • 402
  • 07 Jul 2022
Topic Review
Multiple Neurosyphilitic Maladies
Treponema pallidum (Tp) subspecies pallidum causes syphilis, a sexually transmitted disease that infects more than 2.1 million pregnant women every year. Due to its maximum death rates of neonates, augmented risk of human immunodeficiency virus (HIV) infection, and continued morbidity particularly in low-income countries as well as in high-income countries, such as Japan, where the rate of cases is increasing at an alarming level in heterosexual men and women, syphilis is a disease of worldwide concern, the disease is still a matter of debate in many low- and high-income countries. The infection has three stages that lead to several complications if left untreated and can lead to many tertiary complications in the brain, eyes, ears, heart, and pregnancy. Principally, the infection is transmitted through sexual contact, exceptionally with blood transfusion and blood products, and transmits vertically from mother to child (Syphilis Transmission from Mother-to-Child (MTCT)) during pregnancy. The Tp spirochete transmits vertically to the fetus, leading to congenital syphilis infections in poorly treated or utterly treated pregnant women, and causes multiple clinical manifestations, including stillbirth and neonatal death, skin and visceral manifestations, and other asymptomatic infections. According to the World Health Organization (WHO), a recently published study estimating the burden of congenital syphilis showed more than half a million (almost 661,000) cases of congenital syphilis in 2016, consequently facing 200,000 stillbirths and neonatal deaths. Congenital syphilis is the second leading cause of preventable stillbirth globally, preceded only by “Malaria”. Neurosyphilis (NS) is also known as the clinical result of infection of the central nervous system by Tp subspecies pallidum. It can evolve at any time and from any stage of syphilis exposure. NS involves all neurological disorders related to nervous system invasion by the Tp and can be seen during the primo-secondary (early NS) or tertiary stages. Importantly, NS has two forms: an early form often strikes the CSF, meninges, and vasculature; the late form hits the brain and spinal cord parenchyma, and in several cases, it goes unnoticed or unidentified, leading to multifarious neurological complications.
  • 376
  • 28 Jun 2022
Topic Review
Extracellular Alpha-Synuclein
Alpha-synuclein (α-syn) is a small protein composed of 140 amino acids and belongs to the group of intrinsically disordered proteins. It is a soluble protein that is highly expressed in neurons and expressed at low levels in glial cells. The monomeric protein aggregation process induces the formation of oligomeric intermediates and proceeds towards fibrillar species. These α-syn conformational species have been detected in the extracellular space and mediate consequences on surrounding neurons and glial cells. In particular, higher-ordered α-syn aggregates are involved in microglial and oligodendrocyte activation, as well as in the induction of astrogliosis. These phenomena lead to mitochondrial dysfunction, reactive oxygen and nitrogen species formation, and the induction of an inflammatory response, associated with neuronal cell death. Several receptors participate in cell activation and/or in the uptake of α-syn, which can vary depending on the α-syn aggregated state and cell types. The receptors involved in this process are of outstanding relevance because they may constitute potential therapeutic targets for the treatment of PD and related synucleinopathies.
  • 419
  • 27 Jun 2022
Topic Review
Molecular Hydrogen Neuroprotection in Post-Ischemic Brain Injury
Molecular hydrogen has gained the attention of both preclinical and clinical researchers. The death of pyramidal neurons in especially the CA1 area of the hippocampus, increased permeability of the blood-brain barrier, neuroinflammation, amyloid accumulation, tau protein dysfunction, brain atrophy, cognitive deficits and dementia are considered an integral part of the phenomena occurring during brain neurodegeneration after ischemia.
  • 577
  • 01 Jul 2022
Topic Review
Transgenic Mouse Overexpressing Spermine Oxidase in Cerebrocortical Neurons
Polyamines (PAs) are organic polycations ubiquitously present in living cells. The main PAs in mammalian cells include putrescine (Put), spermidine (Spd), and spermine (Spm), and their acetylated forms, N1-acetylspermidine and N1-acetylspermine. Polyamines are involved in many cellular processes, and their content in mammalian cells is tightly controlled. Among their function, these molecules modulate the activity of several ion channels. Spermine oxidase (SMOX) specifically oxidizes spermine, a neuromodulator of several types of ion channel and ionotropic glutamate receptors, and its deregulated activity has been linked to several brain pathologies, including epilepsy. The Dach-SMOX mouse line was generated using a Cre/loxP-based recombination approach to study the complex and critical functions carried out by spermine oxidase and spermine in the mammalian brain.
  • 418
  • 22 Jun 2022
Topic Review
Immunity, Ion Channels and Epilepsy
Epilepsy is a common chronic neurological disorder in modern society. One of the major unmet challenges is that current antiseizure medications are basically not disease-modifying. Among the multifaceted etiologies of epilepsy, the role of the immune system has attracted considerable attention in recent years. It is known that both innate and adaptive immunity can be activated in response to insults to the central nervous system, leading to seizures. Moreover, the interaction between ion channels, which have a well-established role in epileptogenesis and epilepsy, and the immune system is complex and is being actively investigated.
  • 350
  • 22 Jun 2022
Topic Review
Hibiscus, Rooibos, Yerba Mate in Glycoxidation and Neurodegeneration
The well-known food safety associated with the consumption of hibiscus, rooibos, or yerba mate, and the acceptance of these herbs linked to pleasant taste, have elicited great interest in defining their nutraceutical potential. These plants produce several bioactive metabolites, have a pleasant taste, and a long-lasting history as safe foods. These plants produce several bioactive metabolites, have a pleasant taste, and a long-lasting history as safe foods. The literature on hibiscus, rooibos, and yerba mate teas in the context of nutritional strategies for the attenuation of oxidative stress-related glycoxidation and neurodegeneration was reviewed, and, here, Alzheimer’s Disease is approached as an example. The focus is given to mechanisms of glycation inhibition, as well as neuroprotective in vitro effects, and, in animal studies, to frame interest in these plants as nutraceutical agents related to current health concerns.
  • 579
  • 23 Jun 2022
Topic Review
Neuroendocrine–Immune Regulatory Network of Eucommia ulmoides Oliver
Eucommia ulmoides Oliver (E. ulmoides) is a popular medicinal herb and health supplement in China, Japan, and Korea, and has a variety of pharmaceutical properties. The neuroendocrine–immune (NEI) network is crucial in maintaining homeostasis and physical or psychological functions at a holistic level, consistent with the regulatory theory of natural medicine.
  • 376
  • 22 Jun 2022
Topic Review
Astrocyte Functions
Astrocytes are stellated glial cells that interface with nearly every functional element of the brain. They are the most abundant glial cells in the brain and can comprise up to 50% of the tissue volume in some regions. Their branch-like processes surround neurons, axons, synapses, and blood vessels, and perform numerous functions that are essential for brain homeostasis and neural functioning.
  • 566
  • 17 Jun 2022
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