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Cortical Axonal Secretion of BDNF in the Striatum Is Disrupted in the Mutant-huntingtin Knock-in Mouse Model of Huntington's Disease

Park H

Deficient BDNF signaling is known to be involved in neurodegenerative diseases such as Huntington's disease (HD). Mutant huntingtin (mhtt)-mediated disruption of either BDNF transcription or transport is thought to be...
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Synthetic 3′,4′-Dihydroxyflavone Exerts Anti-Neuroinflammatory Effects in BV2 Microglia and a Mouse Model

Kim N, Yoo HS, Ju YJ, Oh MS, Lee KT, Inn KS, Kim NJ, Lee JK

Neuroinflammation is an immune response within the central nervous system against various proinflammatory stimuli. Abnormal activation of this response contributes to neurodegenerative diseases such as Parkinson disease, Alzheimer’s disease, and...
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Report on the External Quality Assessment Scheme for Molecular Diagnostics in Korea (2017)

Kim MJ, Yoon MH, Song JY, Cho SI, Park SS, Seong MW

Quality control for genetic analysis has become more important with a drastic increase in testing volume and clinical demands. The molecular diagnostics division of the Korean Association of Quality Assurance...
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Quantitative Gait Analysis in Patients with Huntington's Disease

Pyo SJ, Kim H, Kim IS, Park YM, Kim MJ, Lee HM, Koh SB

OBJECTIVE: Gait disturbance is the main factor contributing to a negative impact on quality of life in patients with Huntington’s disease (HD). Understanding gait features in patients with HD is...
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Cell-to-cell Transmission of Polyglutamine Aggregates in C. elegans

Kim DK, Cho KW, Ahn WJ, Perez-Acuña D, Jeong H, Lee HJ, Lee SJ

Huntington disease (HD) is an inherited neurodegenerative disorder characterized by motor and cognitive dysfunction caused by expansion of polyglutamine (polyQ) repeat in exon 1 of huntingtin (HTT). In patients, the...
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Hopping from One Cell to Another: Huntington's Disease Propagates

Park SM

No abstract available.
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Exosome-Based Delivery of miR-124 in a Huntington's Disease Model

Lee ST, Im W, Ban JJ, Lee M, Jung KH, Lee SK, Chu K, Kim M

OBJECTIVE: Huntington's disease (HD) is a genetic neurodegenerative disease that is caused by abnormal CAG expansion. Altered microRNA (miRNA) expression also causes abnormal gene regulation in this neurodegenerative disease. The...
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The Putamen and Caudate Nucleus Volume in Korean Youth by MRI Volumetry

Cho SH, Kwon S, Rhyu IJ

Corpus striatum is subcortical nuclei composed of caudate nucleus and putamen. It has been considered to be associated with motor control and learning. Dysfunction of the striatum is related to...
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Survival of Korean Huntington's Disease Patients

Kim HJ, Shin CW, Jeon B, Park H

OBJECTIVE: The survival of Huntington’s disease (HD) patients is reported to be 15–20 years. However, most studies on the survival of HD have been conducted in patients without genetic confirmation...
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Applications of CRISPR/Cas9 for Gene Editing in Hereditary Movement Disorders

Im W, Moon J, Kim M

Gene therapy is a potential therapeutic strategy for treating hereditary movement disorders, including hereditary ataxia, dystonia, Huntington's disease, and Parkinson's disease. Genome editing is a type of genetic engineering in...
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Psychosis, Treatment Emergent Extrapyramidal Events, and Subsequent Onset of Huntington's Disease: A Case Report and Review of the Literature

Xu C, Yogaratnam J, Tan N, Sim K

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease characterized by a triad of progressive motor dysfunction, cognitive decline and psychiatric disturbances. The hallmark of HD is the distinctive choreiform...
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Neuroprotective effect of caffeic acid phenethyl ester in 3-nitropropionic acid-induced striatal neurotoxicity

Bak J, Kim HJ, Kim SY, Choi YS

Caffeic acid phenethyl ester (CAPE), derived from honeybee hives, is a bioactive compound with strong antioxidant activity. This study was designed to test the neuroprotective effect of CAPE in 3-nitropropionic...
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Annual Report on the External Quality Assessment of Diagnostic Genetics in Korea (2015)

Kim HY, Park CH, Lee SJ, Cho SI, Seong MW, Park SS, Kim SH, Diagnostic Genetics Subcommittee, Korean Association of External Quality Assessment Service

The Diagnostic Genetics Subcommittee of Korean Association of External Quality Assessment Service conducted two trials in 2015 based on cytogenetics and molecular genetics surveys. A total of 43 laboratories participated...
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Combined use of a double-lumen tube and Fogarty catheter to prevent the endobronchial spread of infection: a case report

Kim J, Lee H, Park H, Jeong CY

Huntington's disease is a neurodegenerative disorder with an autosomal dominant inheritance pattern. Patients with Huntington's disease show an increased risk of aspiration pneumonia when the pharyngeal muscle is invaded. We...
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Annual Report on the External Quality Assessment Scheme for Diagnostic Genetics in Korea (2014)

Park CH, Shin SY, Park H, Cho SI, Seong MW, Park SS, Kim SH, Diagnostic Genetics Subcommittee, The Korean Association of External Quality Assessment Service

Quality control for genetic tests has become more important as testing volume and clinical demands have increased dramatically. The diagnostic genetics subcommittee of Korean Association of External Quality Assessment Service...
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Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies

Ciechanover A, Kwon YT

Mammalian cells remove misfolded proteins using various proteolytic systems, including the ubiquitin (Ub)-proteasome system (UPS), chaperone mediated autophagy (CMA) and macroautophagy. The majority of misfolded proteins are degraded by the...
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Stem Cell Therapy for Neurodegenerative Diseases

Yee JZ, Oh KW, Kim SH

Neurodegenerative diseases are the hereditary and sporadic conditions which are characterized by progressive neuronal degeneration. Neurodegenerative diseases are emerging as the leading cause of death, disabilities, and a socioeconomic burden...
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Annual Report on the External Quality Assessment of Diagnostic Genetics in Korea (2013)

Jang MA, Shin SY, Park S, Seong MW, Park SS, Kim SH, Diagnostic Genetics Subcommittee, The Korean Association of Quality Assurance for Clinical Laboratory

  • KMID: 2200443
  • J Lab Med Qual Assur.
  • 2014 Jun;36(2):71-83.
Quality control for genetic tests has become more important as the test volume and clinical demands increase dramatically. The diagnostic genetics subcommittee of the Korean Association of Quality Assurance for...
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In Vivo Roles of a Patient-Derived Induced Pluripotent Stem Cell Line (HD72-iPSC) in the YAC128 Model of Huntington's Disease

Jeon I, Choi C, Lee N, Im W, Kim M, Oh SH, Park IH, Kim HS, Song J

  • KMID: 2054559
  • Int J Stem Cells.
  • 2014 May;7(1):43-47.
Induced pluripotent stem cells (iPSCs) generated from somatic cells of patients can provide immense opportunities to model human diseases, which may lead to develop novel therapeutics. Huntington's disease (HD) is...
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Glutathione Protects Brain Endothelial Cells from Hydrogen Peroxide-Induced Oxidative Stress by Increasing Nrf2 Expression

Song J, Kang SM, Lee WT, Park KA, Lee KM, Lee JE

Glutathione (GSH) protects cells against oxidative stress by playing an antioxidant role. Protecting brain endothelial cells under oxidative stress is key to treating cerebrovascular diseases and neurodegenerative diseases including Alzheimer's...
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