• Title/Summary/Keyword: Peripheral nervous system

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Diagnosis and treatment in Charcot-Marie-Tooth disease (샤르코-마리-투스 질환의 진단 및 치료)

  • Kim, Sang-Beom;Park, Kee Duk;Choi, Byung-Ok
    • Annals of Clinical Neurophysiology
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    • v.7 no.2
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    • pp.65-74
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    • 2005
  • Charcot-Marie-Tooth (CMT) disease was described by Charcot and Marie in France and, independently, by Tooth in England in 1886. CMT is the most common form of inherited motor and sensory neuropathy, and is a genetically heterogeneous disorder of the peripheral nervous system. Therefore, many genes have been identified as CMT-causative genes. Traditionally, subclassification of CMT have been divided into autosomal dominant inherited demyelinating (CMT1) and axonal (CMT2) neuropathies, X-linked neuropathy (CMTX), and autosomal recessive inherited neuropathy (CMT4). Recently, intermediate type (CMT-Int) with NCVs between CMT1 and CMT2 is considered as a CMT type. There are several related peripheral neuropathies, such as $D{\acute{e}}j{\acute{e}}rine$-Sottas neuropathy (DSN), congenital hypomyelination (CH), hereditary neuropathy with liability to pressure palsies (HNPP) and giant axonal neuropathy (GAN). Great advances have been made in understanding the molecular basis of CMT, and 17 distinct genetic causes of CMT have been identified. The number of newly discovered mutations and identified genetic loci is rapidly increasing, and this expanding list has proved challenging for physicians trying to keep up with the field. Identifying the genetic cause of inherited neuropathies is often important to determine at risk family members as well as diagnose the patient. In addition, the encouraging studies have been published on rational potential therapies for the CMT1A. Now, we develop a model of how the various genes may interact in the pathogenesis of CMT disorder.

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Expression of a set of glial cell-specific markers in the Drosophila embryonic central nervous system

  • Ahn, Hui Jeong;Jeon, Sang-Hak;Kim, Sang Hee
    • BMB Reports
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    • v.47 no.6
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    • pp.354-359
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    • 2014
  • The types of glia in the central nervous system (CNS) of the Drosophila embryo include longitudinal glia (LG), cell body glia (CBG), and peripheral glia (PG). Transcription factors, such as glial cell missing and reverse polarity, are well-established general glial cell markers. Only a few glial cell-specific markers have been identified in the Drosophila embryonic CNS, thus far. In the present study, we employed the glial cell-specific markers for LG (vir-1/CG5453 and CG31235), CBG (fabp/CG6783 and CG11902), and PG (CG2310 and moody/CG4322), and comprehensively analyzed their expression patterns, during the embryonic CNS development. Our study validated the specificity of a set of glial markers, and further revealed their spatio-temporal expression patterns, which will aid in the understanding of the developmental lineage, and investigating their role in the development and homeostasis of the Drosophila CNS in vivo.

The Role of the ATP in the Pain Signal Transmission (통증 신호 전달에 있어서 ATP의 역할)

  • Koo Hyun-Mo;Nam Ki-Won;Kim Jin-sang
    • The Journal of Korean Physical Therapy
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    • v.14 no.4
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    • pp.20-27
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    • 2002
  • A role for ATP in nociception and pain induction was proposed. ATP-gated P2X ion channel receptors are localized throughout the nervous system and have been identified on neurons which participate in conduction of nociceptive information from the periphery to central nervous system. We consider the role of ATP as a peripheral activator of nociceptive sensory neuron via ATP-gated ion channels.

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X-linked Charcot-Marie-Tooth disease case with a novel missense mutation in GJB1 gene

  • Lee, Jong-Mok;Shin, Jin-Hong
    • Journal of Genetic Medicine
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    • v.15 no.2
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    • pp.107-109
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    • 2018
  • X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) is caused by the mutation in GJB1 gene, characterized by the transient central nervous system involvement and long standing peripheral polyneuropathy which does not fulfill the criteria of demyelination or axonopathy. We describe a 37-year-old man with progressive bilateral leg weakness since his early teen. He suffered transient right hemiparesis, followed by quadriparesis at 14 years of age. When we examined him at 37 years of age, he presented a distal muscle weakness on lower extremities with a sensory symptom. The nerve conduction study demonstrated a motor conduction velocity between 26 and 49 m/s. The whole exome sequencing revealed a novel variant c.136 G>A in GJB1. This report will raise awareness in this rare disease, which is frequently misdiagnosed early in its course.

Ginsenoside Rg3 reduces the risk of neuronal cell death by attenuating reactive oxygen species and neurotrophins

  • Joo, Seong-Soo;Won, Tae-Joon;Hwang, Kwang-Woo;Lee, Do-Ik
    • Proceedings of the PSK Conference
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    • 2003.10b
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    • pp.134.1-134.1
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    • 2003
  • In regard to $A\beta$ toxicity and AD, reactive oxygen species (ROS) are produced by macrophage families in response to $A\beta$ stimulation. In addition to this, neurotrophins (NTs) regulate the neuronal function as well as cell survival and the growth of various types of neurons in both the peripheral nervous system (PNS) and central nervous system (CNS). As high expressions of the ROS and NTs are a routine findings in neuronal cell damage, we wanted to investigate whether Rg3 can inhibit the production of ROS and NTs primary cell cultures. (omitted)

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Sensory nerve and neuropeptide diversity in adipose tissues

  • Gargi Mishra;Kristy L. Townsend
    • Molecules and Cells
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    • v.47 no.2
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    • pp.100030.1-100030.14
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    • 2024
  • Both brown and white adipose tissues (BAT/WAT) are innervated by the peripheral nervous system, including efferent sympathetic nerves that communicate from the brain/central nervous system out to the tissue, and afferent sensory nerves that communicate from the tissue back to the brain and locally release neuropeptides to the tissue upon stimulation. This bidirectional neural communication is important for energy balance and metabolic control, as well as maintaining adipose tissue health through processes like browning (development of metabolically healthy brown adipocytes in WAT), thermogenesis, lipolysis, and adipogenesis. Decades of sensory nerve denervation studies have demonstrated the particular importance of adipose sensory nerves for brown adipose tissue and WAT functions, but far less is known about the tissue's sensory innervation compared to the better-studied sympathetic nerves and their neurotransmitter norepinephrine. In this review, we cover what is known and not yet known about sensory nerve activities in adipose, focusing on their effector neuropeptide actions in the tissue.

Study of the Relation of Autonomic Nerve System and Peripheral Facial Palsy by the Heart Rate Variability (HRV(Heart Rate Variability)를 통한 말초성 안면신경마비와 자율신경실조의 상관성 연구)

  • Hwang, Gee-Hwae;Hwang, Jong-Soon;Cho, Hyun-Seok;Kim, Kyung-Ho;Kim, Kap-Sung;Lim, Dae-Jung
    • Journal of Acupuncture Research
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    • v.22 no.6
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    • pp.51-60
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    • 2005
  • Objectives : This study was designed to evaluate the effect of inbalance of autonomic nerve system on peripheral facial palsy patients in comparison of Heart Rate Variability results of healthy control group with that of peripheral facial palsy group. Methods : The peripheral facial palsy patiens who visited the Dongguk university Bundang oriental medicine hospital from April to June in 2005 were tested Heart Rate Variability items which are Mean Heart Rate(MHRT), Standard Deviation of all the Normal RR intervals (SDNN), Root Mean Square of Successive Differences Between The Normal Heart Beats(RMSSD), Total Power(TP), Low Frequency, High Frequency, ratio between the Low Frequency and High Frequency power(LF/HF ratio). The Heart Rate Variability test results of peripheral facial palsy group were compared with the HRV test results of healthy control group who didn't have nervous disease, musculoskeletal disease, heart problem and pain for the past 6 months. Results : 1. MHRT was not significantly different between peripheral facial palsy group and healthy control group(P=0.18). 2. SDNN was not significantly different between peripheral facial palsy group and healthy control group(P=0.41). 3. RMSSD was not significantly different between peripheral facial palsy group and healthy control group(P=0.93). 4. TP was not significantly different between peripheral facial palsy group and healthy control group(P=094). 5. LF and HF were not significantly different between peripheral facial palsy group and healthy control group((P=0.34, 0.30). 6. LF/HF ratio was significantly different between peripheral facial palsy group and healthy control group(P=0.04). Conclusion : LF/HF ratio is generally used as autonomic nerve system evaluation and there was significantly statistical difference between peripheral facial palsy group and healthy control group.

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The Effects of Ginsenoside Rg3 as a Potent Inhibitor of Ca2+ Channels and NMDA-gated Channels in the Peripheral and Central Nervous Systems (말초 및 중추신경계에서 칼슘채널 및 NMDA 매개 채널의 억제제로의 진세노사이드 Rg3의 효과)

  • Rhim, Hye-Whon
    • Journal of Ginseng Research
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    • v.27 no.3
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    • pp.120-128
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    • 2003
  • Alternative medicines such as herbal products are increasingly being used for preventive and therapeutic purposes. Ginseng is the best known and most popular herbal medicine used worldwide. In spite of some beneficial effects of ginseng on the nervous system, little scientific evidence shows at the cellular level. In the present study, I have examined the direct modulation of ginseng total saponins and individual ginsenosides on the activation of $Ca^{2+}$ channels and NMDA-gated channels in cultured rat dorsal root ganglion (DRG) and hippocampal neurons, respectively. In DRG neurons, application of ginseng total saponins suppressed high-voltage-activated $Ca^{2+}$ channel currents and ginsenoside Rg$_3$, among the 11 ginsenosides tested, produced the strongest inhibition on $Ca^{2+}$ channel currents. Occlusion experiments using selective $Ca^{2+}$ channel blockers revealed that ginsenoside Rg$_3$ could modulate L-, N-, and P/Q-type currents. In addition, ginsenoside Rg$_3$ also proved to be an active component of ginseng actions on NMDA receptors in cultured hippocampal neurons. Application of ginsenoside Rg$_3$ suppressed NMDA-induced [Ca$^{2+}$]$_{i}$ increase and -gated channels using fura-2-based digital imaging and patch-clamp techniques, respectively. These results suggest that the modulation of $Ca^{2+}$ channels and NMDA receptors by ginsenoside Rg$_3$ could be part of the pharmacological basis of ginseng actions in the peripheral and central nervous systems.ous systems.

Effects of Caffeine on Nerve Conduction Velocity (카페인이 신경전도속도에 미치는 영향)

  • Kang, Yun-Jung
    • Journal of Convergence for Information Technology
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    • v.10 no.3
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    • pp.195-199
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    • 2020
  • This study examined the effect of the nervous-system-stimulating caffeine on the nerve conduction velocity. The purpose of this study is to investigate the effect of caffeine that stimulates the nervous system on nerve conduction test. Although both measurement intervals did not show statistically significant differences when comparing the pre- and post-NCV values within the control and individual experimental groups, it was found that the nerve conductivity in the Axilla-Above Elbow section increased significantly after caffeine intake for the experimental group. Caffeine intake, which has increased the nerve conduction velocity (NCV), was determined to play roles in improving motor skills, muscle strength and nerve performance by temporarily increasing the nerve conduction velocity. Through this study, we learned that caffeine has an influence on the peripheral nervous system as it helps to improve the nerve conduction velocity. upon an appropriate amount of caffeine intake. We hope that these results will help develop treatment and diagnostic methods for patients with nerve dysfunction and myofunctional disorders.

Genetics of Hereditary Peripheral Neuropathies (유전성 말초신경병의 유전학)

  • Cho, Sun-Young;Choi, Byung-Ok
    • Journal of Genetic Medicine
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    • v.6 no.1
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    • pp.25-37
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    • 2009
  • Hereditary peripheral neuropathies can be categorized as hereditary motor and sensory neuropathies (HMSN), hereditary motor neuropathies (HMN), and hereditary sensory neuropathies (HSN). HMSN, HMN, and HSN are further subdivided into several subtypes. Here, we review the most recent findings in the molecular diagnosis and therapeutic strategy for hereditary peripheral neuropathies. The products of genes associated with hereditary peripheral neuropathy phenotypes are important for neuronal structure maintenance, axonal transport, nerve signal transduction, and functions related to the cellular integrity. Identifying the molecular basis of hereditary peripheral neuropathy and studying the relevant genes and their functions is important to understand the pathophysiological mechanisms of these neurodegenerative disorders, as well as the processes involved in the normal development and function of the peripheral nervous system. These advances and the better understanding of the pathogenesis of peripheral neuropathies represent a challenge for the diagnoses and managements of hereditary peripheral neuropathy patients in developing future supportive and curative therapies.

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