• Title/Summary/Keyword: Neurological disorder

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Defective Self-Renewal and Differentiation of GBA-Deficient Neural Stem Cells Can Be Restored By Macrophage Colony-Stimulating Factor

  • Lee, Hyun;Bae, Jae-sung;Jin, Hee Kyung
    • Molecules and Cells
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    • v.38 no.9
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    • pp.806-813
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    • 2015
  • Gaucher disease (GD) is an autosomal recessive lysosomal storage disorder caused by mutations in the glucocerebrosidase gene (GBA), which encodes the lysosomal enzyme glucosylceramidase (GCase). Deficiency in GCase leads to characteristic visceral pathology and lethal neurological manifestations in some patients. Investigations into neurogenesis have suggested that neurodegenerative disorders, such as GD, could be overcome or at least ameliorated by the generation of new neurons. Bone marrowderived mesenchymal stem cells (BM-MSCs) are potential candidates for use in the treatment of neurodegenerative disorders because of their ability to promote neurogenesis. Our objective was to examine the mechanism of neurogenesis by BM-MSCs in GD. We found that neural stem cells (NSCs) derived from a neuronopathic GD model exhibited decreased ability for self-renewal and neuronal differentiation. Co-culture of GBA-deficient NSCs with BM-MSCs resulted in an enhanced capacity for self-renewal, and an increased ability for differentiation into neurons or oligodendrocytes. Enhanced proliferation and neuronal differentiation of GBA-deficient NSCs was associated with elevated release of macrophage colony-stimulating factor (M-CSF) from BM-MSCs. Our findings suggest that soluble M-CSF derived from BM-MSCs can modulate GBA-deficient NSCs, resulting in their improved proliferation and neuronal differentiation.

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

  • Ha, Tai-You
    • IMMUNE NETWORK
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    • v.11 no.5
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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.

TNF-${\alpha}$ Up-regulated the Expression of HuR, a Prognostic Marker for Ovarian Cancer and Hu Syndrome, in BJAB Cells

  • Lee, Kyung-Yeol
    • IMMUNE NETWORK
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    • v.4 no.3
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    • pp.184-189
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    • 2004
  • Background: Hu syndrome, a neurological disorder, is characterized by the remote effect of small cell lung cancer on the neural degeneration. The suspicious effectors for this disease are anti-Hu autoantibodies or Hu-related CD8+ T lymphocytes. Interestingly, the same effectors have been suggested to act against tumor growth and this phenomenon may represent natural tumor immunity. For these diagnostic and therapeutic reasons, the demand for antibodies against Hu protein is rapidly growing. Methods: Polyclonal and monoclonal antibodies were generated using recombinant HuR protein. Western blot analyses were performed to check the specificity of generated antibodies using various recombinant proteins and cell lysates. Extracellular stimuli for HuR expression had been searched and HuR-associated proteins were isolated from polysome lysates and then separated in a 2-dimensional gel. Results: Polyclonal and monoclonal antibodies against HuR protein were generated and these antibodies showed HuR specificity. Antibodies were also useful to detect and immunoprecipitate endogenous HuR protein in Jurkat and BJAB. This report also revealed that TNF-${\alpha}$ treatment in BJAB up-regulated HuR expression. Lastly, protein profile in HuR-associated mRNAprotein complexes was mapped by 2-dimensional gel electrophoresis. Conclusion: This study reported that new antibodies against HuR protein were successfully generated. Currently, project to develop a diagnostic kit is in process. Also, this report showed that TNF-${\alpha}$ up-regulated HuR expression in BJAB and protein profile associated with HuR protein was mapped.

Management Principles of Bilateral Vocal Fold Immobility (양측성 성대 마비의 치료 원칙)

  • Kim, Tae-Wook;Son, Young-Ik
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.20 no.2
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    • pp.118-125
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    • 2009
  • Bilateral vocal fold immobility (BVFI) is a challenging condition which may result from diverse etiologies including vocal fold paralysis, synkinesis, cricoarytenoid joint fixation, and interarytenoid scar. Most patients present with dyspnea and stridor, but sometimes with a breathy dysphonia. Careful history taking, laryngoscopic evaluation under general anesthesia or awaken status, laryngeal EMG, and imaging studies with CT and/or MRI are helpful for providing a precise diagnosis and planning appropriate managements. In children, congenital neurological disorder is one of the most common etiologies, and spontaneous recovery has been reported in more than 50% of cases. Therefore, observation for more than 6 months while securing the upper airway with tracheostomy if needed is a generally accepted rule before deciding any destructive procedure to be undertaken. In children with advanced posterior glottic stenosis, laryngotracheal reconstruction with rib cartilage graft should be considered. In contrast to children, BVFI most commonly occurs as sequalae of surgical complication in adults. Diverse static or dynamic procedures can be applied; posterior cordotomy, vocal fold lateralization, endoscopic or open arytenoidectomy, arytenoid abduction, and reinnervation, electrical laryngeal pacing, which need to be carefully selected according to each patient's needs and pathophysiology of BVFI.

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Analysis of Neurophysiological Approaches to the Intervention of Cerebral palsid child (뇌성마비아 중재에 있어서 신경생리학적 접근기법의 분석)

  • Shin Hong-Cheul;Kang Jung-Koo;Hwang Hwan-Ick;Soe Gyu-Won
    • The Journal of Korean Physical Therapy
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    • v.10 no.1
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    • pp.53-65
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    • 1998
  • The main purpose of this paper is to analyze the modes of therapeutic intervention. The emphasis is on the neurophysiological perpective arising out of neurological principles and developmental concepts. The obtained results are as follows. 1. The important hypostheses predicted that the group intervened by neurodevelopmental approach would improve motor function better than the group done by traditional approach and it was proved that neurodevelopmental approach was more effective in gross motor region(P<.01) 2. In the comparison of type of involovement, neurodevelopmental intervention group in spastic type showed improvements in the region of gross motor.(P<.001) 3. In the comparision of degree of disorder, neurodevelopmental intervention group showed improvement of motor function in all the gross motor region in the mild, moderate and severe case.(P<.001) 4. In the comparison of ages of intervention beginning, the group of child between 25-36, 49-60 and 61-72 months(P<.001) intervened by neurodevelopmental approach showed improvements of motor function. 5. In the comparison of intervention duration, neurodevelopmental intervention group showed improvements of motor function in gross motor region according to intervention durstion(P<.001)

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Foot Drop of Contralateral Limb after Deformity Correction in a Polio Patient: A Case Report

  • Seo, Sang Gyo;Park, Jae Young;Kim, Jin-Tae;Kim, Ji-Beom;Lee, Dong Yeon
    • Journal of Korean Foot and Ankle Society
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    • v.18 no.2
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    • pp.83-86
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    • 2014
  • Postpoliomyelitis syndrome is a common neurological disorder that occurs in patients who have experienced paralytic poliomyelitis. Recently, as a result of vaccination against poliovirus, incidence of poliomyelitis is exceedingly low. However, many patients with postpolio syndrome may encounter anesthesia when undergoing surgery, such as for correction of foot deformity and other operations. We report on a 45-year-old woman who experienced paralysis of her contralateral limb after operation on the left foot under spinal anesthesia. Postoperative electromyography/nerve conduction study (EMG/NCS) was performed in order to determine the cause of paralysis. Motor power of the sequelae involved leg showed improvement with time and recovered fully to the preoperative level at six months after the index operation. A precise evaluation, including a physical examination and EMG/NCS, should be performed preoperatively when spinal anesthesia is planned for postpolio syndrome patients.

Study on the Treatment Mechanism of Back-Shu Points for Organ Dysfunction (배수혈의 내장기 치료 기전에 관한 연구)

  • Hwang, Man-Suk
    • Korean Journal of Acupuncture
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    • v.33 no.3
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    • pp.95-101
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    • 2016
  • Objectives : This study aims to overview the therapeutic mechanism of back-shu points in terms of sympathetic visceral motor nervous system. Methods : Studies about autonomic nervous system, and studies and ancient texts about back-shu points were reviewed. We interpreted possible mechanism of back-shu points considering similarities of anatomical and physiological characteristics of back-shu points and visceral motor nervous system. Results : Afferent signals for organ lesions that can develop the symptoms of autonomic neurological symptoms, pain, hyperalgesia through the skin segment. Through a physical examination of the myotome and dermatome, it is possible to diagnose segmental disorders. Treatment stimulation of the thick fibers of the disorder segment skin can reduce abnormal autonomic influence over the sympathetic reflex mechanism. In addition, if spinal muscles are relaxed, the pressure on the nerve roots could be reduced and consequently the hyperactivity of the sympathetic visceral motor signal would be suppressed. Conclusions : The back-shu points treatments work through the mechanism of the sympathetic nervous reflex. Moreover, segmental acupuncture can reduce tension of the spinal muscles, thereby improving pathological conditions of the sympathetic nervous system.

A case study of tremor patient with Yeokgansan-gamibang (억간산(抑肝散) 가미방(加味方)으로 증상 호전 보인 진전 환자 1례에 대한 증례 보고)

  • Lim Seong-Min;An Joung-Jo;Choi Byung-Ryul;Song Gi-Chul;Kim Man-Ho;Kim Yun-Sik;Seol In-Chan
    • The Journal of Internal Korean Medicine
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    • v.24 no.4_2
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    • pp.1062-1066
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    • 2003
  • Tremor is a rhythmic and involuntary muscular contraction characterized by oscillations of body parts. Tremor often accompanies neurological disorders associated with aging. Although the disorder is not life-threatening, it can be responsible for functional disability and interpersonal relationship problems. We have recently examined a number of tremor-patients. one of them was improved significantly through the herbal medicine (Yeokgansan-gamibang) and acupuncture, so we report it for the better treatment.

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Attenuated Cerebral Ischemic Injury by Polyethylene Glycol-Conjugated Hemoglobin

  • Cho, Geum-Sil;Choi, In-Young;Choi, Yoo-Keum;Kim, Seul-Ki;Cai, Ying;Nho, Kwang;Lee, Jae-Chul
    • Biomolecules & Therapeutics
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    • v.17 no.3
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    • pp.270-275
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    • 2009
  • Polyethylene glycol-conjugated hemoglobin (PEG-Hb) has been proposed as a blood substitute for transfusion due to their plasma expansion and oxygen transport capabilities. The protective effect of PEG-Hb on cerebral hypoxic-ischemic injury was investigated in neonatal hypoxia model and adult rat focal cerebral ischemia model. As intravenously administered 30 min before the onset of hypoxia, PEG-Hb markedly protected cerebral hypoxic injury in a neonatal rat hypoxia model. A similar treatment of PEG-Hb largely reduced the ischemic injury ensuing after 2-h middle cerebral artery occlusion followed by 22-h reperfusion. Consistently, neurological disorder was significantly improved by PEG-Hb. The results indicate that the pharmacological blockade of cerebral ischemic injury by using PEG-Hb may provide a useful strategy for the treatment of cerebral stroke.

A Clinical Study on 4 Cases of Tremor Patients with Acupuncture Treatment (침(針)과 한약(韓藥) 치료(治療)를 통한 진전 환자 4례(例)에 대한 증례보고)

  • Shin, Hyun-Kwon;Kong, Hyun-Woo;Park, Se-Jin;Seo, Joo-Hui;Kim, Joo-Won
    • Journal of Oriental Neuropsychiatry
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    • v.16 no.2
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    • pp.267-276
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    • 2005
  • Tremor is a rhythmic, involuntary muscular contraction characterized by oscillations(to-and-fro movements) of a part of the body. The most common of all involuntary movements, tremor can affect various body parts such as the hands, head, facial structures, vocal cords, trunk, and legs; most tremors, however, occur in the hands. Tremor often accompanies neurological disorders associated with aging. Although the disorder is not life-threatening, it can be responsible for functional disability and social embarrassment. The clinical study was carried out the 4 patients with tremor who were treated in Dong Seo Medical Center from 1 January 2005 to 31 August. The results were summarized as follows. Tremor has relation to liver according to Jangbu theory. So we performed acupuncture treatment(Ganseonggyeok) and Herbal medication on tremor. After our treatments, tremor and some other symptoms were improved.

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