• 제목/요약/키워드: Neuromuscular junction diseases

검색결과 5건 처리시간 0.019초

Therapeutic Potential of CKD-504, a Novel Selective Histone Deacetylase 6 Inhibitor, in a Zebrafish Model of Neuromuscular Junction Disorders

  • Jeong, Hui Su;Kim, Hye Jin;Kim, Deok-Ho;Chung, Ki Wha;Choi, Byung-Ok;Lee, Ji Eun
    • Molecules and Cells
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    • 제45권4호
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    • pp.231-242
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    • 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.

Application of tenotomy on Korean native cattle (Hanwoo) with spastic paresis symptoms in the field

  • Younghye Ro ;Woojae Choi;Leegon Hong ;Kyunghyun Min;Inkwang Ryu;Danil Kim
    • Journal of Veterinary Science
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    • 제24권3호
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    • pp.45.1-45.6
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    • 2023
  • Bovine spastic paresis (BSP) is a neuromuscular disorder characterized by hypertension and stiffness of hindlimb. Two Korean native cattle (Hanwoo) calves developed BSP or BSP-like symptoms, and a tenotomy of superficial tendon of medial head and deep tendon of lateral head of gastrocnemius muscle was performed for treatment. A cast was applied postoperatively to prevent muscle rupture and was removed three weeks later. The prognosis was evaluated at 3 weeks, 6 and 18 months postoperatively. Neither calf showed any other postoperative sequelae. This is the first case study to report the diagnosis, treatment, and prognosis of BSP in Hanwoo.

미각 이상 환자에서의 후기 눈깜박 반사 검사 이상소견: 증례보고 (Late Blink Reflex Abnormality in a Patient with Dysgeusia: A Case Report)

  • 박홍범;한아름;김기훈;박병규;김동휘
    • 대한근전도전기진단의학회지
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    • 제20권2호
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    • pp.144-147
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    • 2018
  • Although dysgeusia can occur as a consequence of stroke attacks, many physicians and patients tend to overlook it. A 50-year old woman complained of a 2-week history of abnormal sense of taste on the anterior two-thirds of right tongue. Blink reflex test demonstrated prolonged ipsilateral and contralateral R2 responses with the right supraorbital nerve stimulations, which suggest the lesion on the descending pathway. Brainstem magnetic resonance imaging (MRI) demonstrated abnormal findings in the right lower dorsal pons, anterior to 4th ventricle, lateral to inferior colliculus, and at the level of the pontomedullary junction, which was compatible with solitary tract nucleus and spinal trigeminal nucleus. Brainstem infarction should be considered in patients who have abnormal sense of taste. Additionally, blink reflex test may be helpful for the detection of central origin dysgeusia.

Occupational Neurotoxic Diseases in Taiwan

  • Liu, Chi-Hung;Huang, Chu-Yun;Huang, Chin-Chang
    • Safety and Health at Work
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    • 제3권4호
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    • pp.257-267
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    • 2012
  • Occupational neurotoxic diseases have become increasingly common in Taiwan due to industrialization. Over the past 40 years, Taiwan has transformed from an agricultural society to an industrial society. The most common neurotoxic diseases also changed from organophosphate poisoning to heavy metal intoxication, and then to organic solvent and semiconductor agent poisoning. The nervous system is particularly vulnerable to toxic agents because of its high metabolic rate. Neurological manifestations may be transient or permanent, and may range from cognitive dysfunction, cerebellar ataxia, Parkinsonism, sensorimotor neuropathy and autonomic dysfunction to neuromuscular junction disorders. This study attempts to provide a review of the major outbreaks of occupational neurotoxins from 1968 to 2012. A total of 16 occupational neurotoxins, including organophosphates, toxic gases, heavy metals, organic solvents, and other toxic chemicals, were reviewed. Peer-reviewed articles related to the electrophysiology, neuroimaging, treatment and long-term follow up of these neurotoxic diseases were also obtained. The heavy metals involved consisted of lead, manganese, organic tin, mercury, arsenic, and thallium. The organic solvents included n-hexane, toluene, mixed solvents and carbon disulfide. Toxic gases such as carbon monoxide, and hydrogen sulfide were also included, along with toxic chemicals including polychlorinated biphenyls, tetramethylammonium hydroxide, organophosphates, and dimethylamine borane. In addition we attempted to correlate these events to the timeline of industrial development in Taiwan. By researching this topic, the hope is that it may help other developing countries to improve industrial hygiene and promote occupational safety and health care during the process of industrialization.

소세포폐암환자에서 진단된 VGCC 항체 양성인 Lambert-Eaton 근무력 증후군 1예 (A Case of Lambert-Eaton Myasthenic Syndrome with Positive VGCC Antibodies Diagnosed in Small Cell Lung Cancer)

  • 김소영;오효정;황기은;정종훈;김학렬;양세훈;조광호;정은택
    • Tuberculosis and Respiratory Diseases
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    • 제59권4호
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    • pp.427-431
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    • 2005
  • 저자들은 점점 진행하는 하지무력증을 주소로 내원한 48세 남자에서 전기생리검사를 시행하여 VGCC 양성인 LEMS를 진단하였고, 동반한 소세포폐암을 진단하여 항암요법을 시행하였고 완치 판정을 받은 후 특별한 치료 없이도 LEMS의 임상증상과 전기생리검사에서 호전을 보인 1예를 경험하였기에 이를 보고하는 바이다.