• Molecules and Cells
• /
• v.43 no.3
• /
• pp.203-214
• /
• 2020

Post-translational modifications play major roles in the stability, function, and localization of target proteins involved in the nervous system. The ubiquitin-proteasome pathway uses small ubiquitin molecules to degrade neuronal proteins. Deubiquitinating enzymes (DUBs) reverse this degradation and thereby control neuronal cell fate, synaptic plasticity, axonal growth, and proper function of the nervous system. Moreover, mutations or downregulation of certain DUBs have been found in several neurodegenerative diseases, as well as gliomas and neuroblastomas. Based on emerging findings, DUBs represent an important target for therapeutic intervention in various neurological disorders. Here, we summarize advances in our understanding of the roles of DUBs related to neurobiology.

• Mass Spectrometry Letters
• /
• v.14 no.3
• /
• pp.57-78
• /
• 2023

Understanding the chemical composition of the brain helps researchers comprehend various neurological processes effectively. Understanding of the fundamental pathological processes that underpin many neurodegenerative disorders has recently advanced thanks to the advent of innovative bioanalytical techniques that allow high sensitivity and specificity with chemical imaging at high resolution in tissues and cells. Mass spectrometry imaging [MSI] has become more common in biomedical research to map the spatial distribution of biomolecules in situ. The technique enables complete and untargeted delineation of the in-situ distribution characteristics of proteins, metabolites, lipids, and peptides. MSI's superior molecular specificity gives it a significant edge over traditional histochemical methods. Recent years have seen a significant increase in MSI, which is capable of simultaneously mapping the distribution of thousands of biomolecules in the tissue specimen at a high resolution and is otherwise beyond the scope of other molecular imaging techniques. This review aims to acquaint the reader with the MSI experimental workflow, significant recent advancements, and implementations of MSI techniques in visualizing the anatomical distribution of neurochemicals in the human brain in relation to various neurogenerative diseases.

• Molecules and Cells
• /
• v.45 no.4
• /
• pp.231-242
• /
• 2022

The neuromuscular junction (NMJ), which is a synapse for signal transmission from motor neurons to muscle cells, has emerged as an important region because of its association with several peripheral neuropathies. In particular, mutations in GARS that affect the formation of NMJ result in Charcot-Marie-Tooth disease and distal hereditary motor neuropathy. These disorders are mainly considered to be caused by neuronal axon abnormalities; however, no treatment is currently available. Therefore, in order to determine whether the NMJ could be targeted to treat neurodegenerative disorders, we investigated the NMJ recovery effect of HDAC6 inhibitors, which have been used in the treatment of several peripheral neuropathies. In the present study, we demonstrated that HDAC6 inhibition was sufficient to enhance movement by restoring NMJ impairments observed in a zebrafish disease model. We found that CKD-504, a novel HDAC6 inhibitor, was effective in repairing NMJ defects, suggesting that treatment of neurodegenerative diseases via NMJ targeting is possible.

• CELLMED
• /
• v.2 no.1
• /
• pp.2.1-2.11
• /
• 2012

Tea polyphenols especially catechins have long been studied for their antioxidant and radical scavenging properties. Scientists throughout the world have investigated the usefulness of the regular green tea consumption in several disease conditions. In-vitro and in-vivo experiments on catechins especially epigallocatechingallate have revealed a significant role in many ways. Reactive oxygen species have been increasingly implicated in the pathogenesis of many diseases and important biological processes. Toxic effects of these oxidants, commonly referred to as oxidative stress, can cause cellular damage by oxidizing nucleic acids, proteins, and membrane lipids. Oxidative stress has been related to aging and age related disorders. It is found that in a wide variety of pathological processes, including cancer, atherosclerosis, neurological degeneration, Alzheimer's disease, ageing and autoimmune disorders, oxidative stress has its implications. Catechins have been reported to be useful in combating aging and age related disorders like cancer, cardiovascular disorders and neurodegenerative diseases. In this mini review we will discuss such studies done across the globe.

• Molecular & Cellular Toxicology
• /
• v.3 no.2
• /
• pp.77-84
• /
• 2007

Millions of individuals worldwide are currently afflicted with neurodegenerative disorders such as Parkinson's disease and multiple sclerosis which are caused by the death of specific types of specialized cells in the Central Nervous System (CNS). Recently, Neural Stem Cells (NSCs) are able to replace these dead cells with new functional cells, thereby providing a cure for devastating neural diseases. The clinical use of neural stem cells (NSCs) for the treatment of neurological diseases requires overcoming the scarcity of the initial in vivo NSC population. Thus, we developed a novel 3-dimentional cellular automata model for optimum production of neural stem cells and their derivatives in large scale to treat neurodegenerative disorder patients.

• Journal of Ginseng Research
• /
• v.36 no.4
• /
• pp.342-353
• /
• 2012

Ginseng, the root of the Panax ginseng, has been a popular and widely-used traditional herbal medicine in Korea, China, and Japan for thousands of years. Now it has become popular as a functional health food and is used globally as a natural medicine. Evidence is accumulating in the literature on the physiological and pharmacological effects of P. ginseng on neurodegenerative diseases. Possible ginseng- or ginsenosides-mediated neuroprotective mechanisms mainly involve maintaining homeostasis, and anti-inflammatory, anti-oxidant, anti-apoptotic, and immune-stimulatory activities. This review considers publications dealing with the various actions of P. ginseng that are indicative of possible neurotherapeutic efficacies in neurodegenerative diseases and neurological disorders such as Parkinson's disease, Alzheimer's disease, Huntington's disease, and amyotrophic lateral sclerosis and multiple sclerosis.

• Journal of Genetic Medicine
• /
• v.18 no.1
• /
• pp.16-23
• /
• 2021

GLB1-related disorders comprise two phenotypically unique disorders: GM1 gangliosidosis and Morquio B disease. These autosomal recessive disorders are caused by b-galactosidase deficiency. A hallmark of GM1 gangliosidosis is central nervous system degeneration where ganglioside synthesis is highest. The accumulation of keratan sulfate is the suspected cause of the bone findings in Morquio B disease. GM1 gangliosidosis is clinically characterized by a neurodegenerative disorder associated with dysostosis multiplex, while Morquio B disease is characterized by severe skeletal manifestations and the preservation of intelligence. Morquio B disease and GM1 gangliosidosis may be on a continuum of skeletal involvement. There is currently no effective treatment for GLB1-related disorders. Recently, multiple interventions have been developed and there are several ongoing clinical trials.

• Proceedings of the PSK Conference
• /
• 2002.10a
• /
• pp.324.2-325
• /
• 2002

Recent studies suggest that inflammatory events are implicated in a variety of human diseases including cancer and neurodegenerative diseases. and non-steroidal anti-inflammatory drugs have beneficial effects in treatment or prevention of these disorders. It has been reported that expression of cyclooxygenase (COX)-2 and nitric oxide synthase and subsequent production of prostaglandin (PG) and nitric oxide (NO). respectively are elevated in many inflammatory disorders. (omitted)

• Development and Reproduction
• /
• v.13 no.2
• /
• pp.63-77
• /
• 2009

The neuronal ceroid-lipofuscinoses (NCLs) are a kind of neurodegenerative storage disorders. The NCLs are charecterizated by accumulation of autofluorescent lipofuscin or lipopigment in the brain. All NCL group belongs to in lysosomal storage disorders (LSDs), except Northern epilepsy. NCLs are the most common group of progressive neurodegenerative disorders in childhood, with an incidence as high as I in 12,500 live births. Four main clinical types have been described based on the onset age : infantile, late infantile, juvenile and adult types. Clinical symptoms of NCLs include loss of vision, seizures, epilepsy, progressive mental retardation and a premature death. Although mutation causes neurodegeneration in NCLs, the molecular mechanism by which mutation leads to neurodegeneration remains unclear. In this paper, we review the characteristics of these NCLs.

• BMB Reports
• /
• v.53 no.10
• /
• pp.500-511
• /
• 2020

14-3-3 proteins are mostly expressed in the brain and are closely involved in numerous brain functions and various brain disorders. Among the isotypes of the 14-3-3 proteins, 14-3-3γ is mainly expressed in neurons and is highly produced during brain development, which could indicate that it has a significance in neural development. Furthermore, the distinctive levels of temporally and locally regulated 14-3-3γ expression in various brain disorders suggest that it could play a substantial role in brain plasticity of the diseased states. In this review, we introduce the various brain disorders reported to be involved with 14-3-3γ, and summarize the changes of 14-3-3γ expression in each brain disease. We also discuss the potential of 14-3-3γ for treatment and the importance of research on specific 14-3-3 isotypes for an effective therapeutic approach.