• 제목/요약/키워드: Huntington disease

검색결과 45건 처리시간 0.028초

New insight into transglutaminase 2 and link to neurodegenerative diseases

  • Min, Boram;Chung, Kwang Chul
    • BMB Reports
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    • 제51권1호
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    • pp.5-13
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    • 2018
  • Formation of toxic protein aggregates is a common feature and mainly contributes to the pathogenesis of neurodegenerative diseases (NDDs), which include amyotrophic lateral sclerosis (ALS), Alzheimer's, Parkinson's, Huntington's, and prion diseases. The transglutaminase 2 (TG2) gene encodes a multifunctional enzyme, displaying four types of activity, such as transamidation, GTPase, protein disulfide isomerase, and protein kinase activities. Many studies demonstrated that the calcium-dependent transamidation activity of TG2 affects the formation of insoluble and toxic amyloid aggregates that mainly consisted of NDD-related proteins. So far, many important and NDD-related substrates of TG2 have been identified, including $amlyoid-{\beta}$, tau, ${\alpha}-synuclein$, mutant huntingtin, and ALS-linked trans-activation response (TAR) DNA-binding protein 43. Recently, the formation of toxic inclusions mediated by several TG2 substrates were efficiently inhibited by TG2 inhibitors. Therefore, the development of highly specific TG2 inhibitors would be an important tool in alleviating the progression of TG2-related brain disorders. In this review, the authors discuss recent advances in TG2 biochemistry, several mechanisms of molecular regulation and pleotropic signaling functions, and the presumed role of TG2 in the progression of many NDDs.

Minocycline Directly Blocks Activation of Caspases After Oxidative Stress in PC12 Cells

  • Choi, Yu-Keum;Kim, Gab-Seok;Han, Byung-Hee
    • 대한약학회:학술대회논문집
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    • 대한약학회 2003년도 Proceedings of the Convention of the Pharmaceutical Society of Korea Vol.2-2
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    • pp.79.2-79.2
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    • 2003
  • Minocycline is known to protect neurons from microglia-mediated cell death in many experimental models of brain diseases including ischemic stroke, Huntington's disease (HD), amyotrophic lateral sclerosis (ALS), traumatic brain injury, multiple sclerosis, and Parkinson's disease. Activation of caspase-2, 3, 8, and 9 was evident within 2-8 hr following oxidative insult with 0.5 mM hydrogen peroxide in PC12 cells. Minocycline significantly attenuated activation of these caspases up to 18 hr, resulting a significant increase in cell viability as assessed by MTT assay. (omitted)

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Synapses in neurodegenerative diseases

  • Bae, Jae Ryul;Kim, Sung Hyun
    • BMB Reports
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    • 제50권5호
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    • pp.237-246
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    • 2017
  • Synapse is the basic structural and functional component for neural communication in the brain. The presynaptic terminal is the structural and functionally essential area that initiates communication and maintains the continuous functional neural information flow. It contains synaptic vesicles (SV) filled with neurotransmitters, an active zone for release, and numerous proteins for SV fusion and retrieval. The structural and functional synaptic plasticity is a representative characteristic; however, it is highly vulnerable to various pathological conditions. In fact, synaptic alteration is thought to be central to neural disease processes. In particular, the alteration of the structural and functional phenotype of the presynaptic terminal is a highly significant evidence for neural diseases. In this review, we specifically describe structural and functional alteration of nerve terminals in several neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), Amyotrophic lateral sclerosis (ALS), and Huntington's disease (HD).

Effects of Red ginseng on neuroinflammation in neurodegenerative diseases

  • Min Yeong Lee;Mikyung Kim
    • Journal of Ginseng Research
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    • 제48권1호
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    • pp.20-30
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    • 2024
  • Red ginseng (RG) is widely used as a herbal medicine. As the human lifespan has increased, numerous diseases have developed, and RG has also been used to treat various diseases. Neurodegenerative diseases are major problems that modern people face through their lives. Neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, Huntington's disease, and amyotrophic lateral sclerosis are featured by progressive nerve system damage. Recently, neuroinflammation has emerged as a degenerative factor and is an immune response in which cytokines with nerve cells that constitute the nervous system. RG, a natural herbal medicine with fewer side effects than chemically synthesized drugs, is currently in the spotlight. Therefore, we reviewed studies reporting the roles of RG in treating neuroinflammation and neurodegenerative diseases and found that RG might help alleviate neurodegenerative diseases by regulating neuroinflammation.

Animal Models of Cognitive Deficits for Probiotic Treatment

  • Kwon, Oh Yun;Lee, Seung Ho
    • 한국축산식품학회지
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    • 제42권6호
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    • pp.981-995
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    • 2022
  • Cognitive dysfunction is a common symptom of neurodegenerative diseases, such as Alzheimer's disease, Parkinson's disease, and Huntington's disease, and is known to be caused by the structural and functional loss of neurons. Many natural agents that can improve cognitive function have been developed and assessed for efficacy using various cognitive deficit animal models. As the gut environment is known to be closely connected to brain function, probiotics are attracting attention as an effective treatment target that can prevent and mitigate cognitive deficits as a result of neurodegenerative diseases. Thus, the objective of this review is to provide useful information about the types and characteristics of cognitive deficit animal models, which can be used to evaluate the anti-cognitive effects of probiotics. In addition, this work reviewed recent studies describing the effects and treatment conditions of probiotics on cognitive deficit animal models. Collectively, this review shows the potential of probiotics as edible natural agents that can mitigate cognitive impairment. It also provides useful information for the design of probiotic treatments for cognitive deficit patients in future clinical studies.

MicroRNAs in Human Diseases: From Autoimmune Diseases to Skin, Psychiatric and Neurodegenerative Diseases

  • Ha, Tai-You
    • IMMUNE NETWORK
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    • 제11권5호
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    • pp.227-244
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    • 2011
  • MicroRNAs (miRNAs) are small noncoding RNA molecules that negatively regulate gene expression via degradation or translational repression of their target messenger RNAs (mRNAs). Recent studies have clearly demonstrated that miRNAs play critical roles in several biologic processes, including cell cycle, differentiation, cell development, cell growth, and apoptosis and that miRNAs are highly expressed in regulatory T (Treg) cells and a wide range of miRNAs are involved in the regulation of immunity and in the prevention of autoimmunity. It has been increasingly reported that miRNAs are associated with various human diseases like autoimmune disease, skin disease, neurological disease and psychiatric disease. Recently, the identification of miRNAs in skin has added a new dimension in the regulatory network and attracted significant interest in this novel layer of gene regulation. Although miRNA research in the field of dermatology is still relatively new, miRNAs have been the subject of much dermatological interest in skin morphogenesis and in regulating angiogenesis. In addition, miRNAs are moving rapidly center stage as key regulators of neuronal development and function in addition to important contributions to neurodegenerative disorder. Moreover, there is now compelling evidence that dysregulation of miRNA networks is implicated in the development and onset of human neruodegenerative diseases, such as Alzheimer's disease, Parkinson's disease, Huntington's disease, Tourette's syndrome, Down syndrome, depression and schizophrenia. In this review, I briefly summarize the current studies about the roles of miRNAs in various autoimmune diseases, skin diseases, psychoneurological disorders and mental stress.

A proteomic approach to identify of yeast proteins that related with accumulation of misfolded protein in cell

  • Shin, Yong-Seung;Seo, Eun-Joo;Kim, Joon;Yu, Myeong-Hee
    • 한국생물물리학회:학술대회논문집
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    • 한국생물물리학회 2003년도 정기총회 및 학술발표회
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    • pp.64-64
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    • 2003
  • In growing number of diseases it has been shown that the aggregation of specific proteins has an important role in the pathogenesis of the disorder. This has been demonstrated in structural detail with the liver cirrhosis of ${\alpha}$$_1$-antitrypsin deficiency, and it is now believed that similar protein aggregation underlies many neurodegenerative disorders such as autosomal dominant Parkinson disease, prion diseases, Alzheimer disease, Huntington disease.

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A proteomic approach to identify yeast proteins responding to accumulation of misfolded proteins inside the cells

  • Shin, Yong-Seung;Seo, Eun-Joo;Kim, Joon;Yu, Myeong-Hee
    • 한국생물물리학회:학술대회논문집
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    • 한국생물물리학회 2003년도 정기총회 및 학술발표회
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    • pp.57-57
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    • 2003
  • In growing number of diseases it has been shown that aggregation of specific proteins has an important role in pathogenesis of the disorder. This has been demonstrated in structural details with the liver cirrhosis of ${\alpha}$$_1$-antitrypsin deficiency, and it is now believed that similar protein aggregation underlies many neurodegenerative disorders such as autosomal dominant Parkinson disease, prion diseases, Alzheimer disease, and Huntington disease. ${\alpha}$$_1$-Antieypsin, a member of serine pretense inhibitor (serpin) family, functions as an inhibitor of neutrophil elastase.

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Effects of Panax ginseng in Neurodegenerative Diseases

  • Cho, Ik-Hyun
    • Journal of Ginseng Research
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    • 제36권4호
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    • pp.342-353
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    • 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.