1.A Case of Thyrotoxic Myopathy with Extreme Type 2 Fiber Predominance.
Tae Jung KIM ; Han Sang LEE ; Je Young SHIN ; Dong Gun KIM ; Sung Min KIM ; Jung Joon SUNG ; Kwang Woo LEE
Experimental Neurobiology 2013;22(3):232-234
In hyperthyroidism, many patients had neuromuscular symptoms and clinical weakness correlated with free thyroxine (T4) concentrations. The common clinical symptoms of chronic thyrotoxic myopathy were characterized by progressive weakness in proximal muscles and atrophy. A 55-year old woman was visited our hospital with two years of progressive weakness of both legs. Physical examination showed diffuse enlargement of the thyroid gland, muscle atrophy and tachycardia. Motor examination showed proximal weakness in both legs. Serum creatine phosphokinase was normal and electromyography showed a myopathic pattern. Serum thyroxine (T4) was greatly increased and serum thyroid stimulating hormone was very low. Muscle biopsy showed mild atrophic change and type 2 fiber predominance. The patient's symptoms were improved during treatment with methimazole. Herein we report a case of thyrotoxic myopathy with extreme type 2 fiber predominance histologically.
Atrophy
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Biopsy
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Creatine Kinase
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Electromyography
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Female
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Humans
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Hyperthyroidism
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Leg
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Methimazole
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Muscles
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Muscular Atrophy
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Muscular Diseases
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Physical Examination
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Tachycardia
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Thyroid Gland
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Thyrotropin
;
Thyroxine
2.Cocaine-Induced Behavioral Sensitization in Mice: Effects of Microinjection of Dopamine D2 Receptor Antagonist into the Nucleus Accumbens.
Eun Sol JUNG ; Hyo Jin LEE ; Hye Ri SIM ; Ja Hyun BAIK
Experimental Neurobiology 2013;22(3):224-231
To determine the role of dopamine D2 receptor (D2R) in the nucleus accumbens (NAc) core in cocaine-induced behavioral sensitization, D2R antagonist, raclopride was bilaterally microinjected (2.5 or 5 nmol) into the NAc core of WT and D2R-/- mice and the initiation and expression phase of cocaine-mediated locomotor sensitization were analyzed. WT and D2R knockout (D2R-/-) mice received bilateral injections of either saline, or raclopride at the NAc core 30 min before each of five daily repeated injections of saline or cocaine (15 mg/kg i.p.). Following 2 weeks of withdrawal after repeated exposure to cocaine, the animals were pre-treated with an intra-accumbal injection of vehicle or raclopride before receiving a systemic cocaine challenge for the expression of sensitization. Animals which had been microinjected raclopride into NAc core displayed the enhancement of cocaine-induced behavioral response for the initiation but also for the expression of sensitization in WT as well as in D2R-/- mice, which was thus unaltered as compared to vehicle-injected control group. These results suggest that D2R in NAc core is not involved in cocaine-induced behavioral sensitization.
Animals
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Cocaine
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Dopamine
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Mice
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Microinjections
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Nucleus Accumbens
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Raclopride
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Receptors, Dopamine
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Receptors, Dopamine D2
3.Erratum: Neuroprotective Effect of Lucium chinense Fruit on Trimethyltin-Induced Learning and Memory Deficits in the Rats.
Hyun Jung PARK ; Hyun Soo SHIM ; Woong Ki CHOI ; Kyung Soo KIM ; Insop SHIM
Experimental Neurobiology 2011;20(4):197-197
No abstract available.
4.Odor-Dependent Hemodynamic Responses Measured with NIRS in the Main Olfactory Bulb of Anesthetized Rats.
Hyun Joo LEE ; Yunjun NAM ; Chin Su KOH ; Changkyun IM ; In Seok SEO ; Seungjin CHOI ; Hyung Cheul SHIN
Experimental Neurobiology 2011;20(4):189-196
In this study, we characterize the hemodynamic changes in the main olfactory bulb of anesthetized Sprague-Dawley (SD) rats with near-infrared spectroscopy (NIRS, ISS Imagent) during presentation of two different odorants. Odorants were presented for 10 seconds with clean air via an automatic odor stimulator. Odorants are: (i) plain air as a reference (Blank), (ii) 2-Heptanone (HEP), (iii) Isopropylbenzene (IB). Our results indicated that a plain air did not cause any change in the concentrations of oxygenated (Delta[HbO2]) and deoxygenated hemoglobin (Delta[Hbr]), but HEP and IB induced strong changes. Furthermore, these odor-specific changes had regional differences within the MOB. Our results suggest that NIRS technology might be a useful tool to identify of various odorants in a non-invasive manner using animals which has a superb olfactory system.
Animals
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Benzene Derivatives
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Hemodynamics
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Hemoglobins
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Ketones
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Odors
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Olfactory Bulb
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Oxygen
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Rats
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Spectroscopy, Near-Infrared
5.Transmission of Synucleinopathies in the Enteric Nervous System of A53T Alpha-Synuclein Transgenic Mice.
He Jin LEE ; Ji Eun SUK ; Kyung Won LEE ; Seung Hwa PARK ; Peter C BLUMBERGS ; Wei Ping GAI ; Seung Jae LEE
Experimental Neurobiology 2011;20(4):181-188
Parkinson's disease (PD) and dementia with Lewy bodies (DLB) are characterized by abnormal deposition of alpha-synuclein aggregates in many regions of the central and peripheral nervous systems. Accumulating evidence suggests that the alpha-synuclein pathology initiates in a few discrete regions and spreads to larger areas in the nervous system. Recent pathological studies of PD patients have raised the possibility that the enteric nervous system is one of the initial sites of alpha-synuclein aggregation and propagation. Here, we evaluated the induction and propagation of alpha-synuclein aggregates in the enteric nervous system of the A53T alpha-synuclein transgenic mice after injection of human brain tissue extracts into the gastric walls of the mice. Western analysis of the brain extracts showed that the DLB extract contained detergent-stable alpha-synuclein aggregates, but the normal brain extract did not. Injection of the DLB extract resulted in an increased deposition of alpha-synuclein in the myenteric neurons, in which alpha-synuclein formed punctate aggregates over time up to 4 months. In these mice, inflammatory responses were increased transiently at early time points. None of these changes were observed in the A53T mice injected with saline or the normal brain extract, nor were these found in the wild type mice injected with the DLB extract. These results demonstrate that pathological alpha-synuclein aggregates present in the brain of DLB patient can induce the aggregation of endogenous alpha-synuclein in the myenteric neurons in A53T mice, suggesting the transmission of synucleinopathy lesions in the enteric nervous system.
alpha-Synuclein
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Animals
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Brain
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Dementia
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Enteric Nervous System
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Humans
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Inflammation
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Lewy Bodies
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Mice
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Mice, Transgenic
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Nervous System
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Neurons
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Parkinson Disease
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Peripheral Nervous System
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Tissue Extracts
6.Effect of Strychinine, a Glycine Inhibitor, on the Programmed Cell Death of Motoneurons during the Chick Development.
Joo Yeon KIM ; So Yoen CHOI ; Hyun KIM ; Woong SUN
Experimental Neurobiology 2011;20(4):176-180
In this study, we report that the treatment of strychinine (STR), an inhibitor of glycine receptor, induced premature onset of programmed cell death (PCD) of developing chick motoneurons (MNs). Treatment of STR on E4 chick embryo increased the apoptosis of MN on E5 when MN PCD does not occur normally. On the other hand, treatment of STR from E3 or E5 for 24 hours did not significantly influence the extent of MN PCD, indicating that the STR effect is developmental stage-specific. However, the expression of glycine receptor isoform was low on E3-4, and other glycine receptor antagonists did not exhibit PCD-promoting activity, suggesting that the STR action on PCD is not related to the glycine receptor activation. Identification of the target molecule for STR action may provide novel mechanism how the onset of developmental PCD is regulated.
Animals
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Apoptosis
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Cell Death
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Chick Embryo
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Glycine
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Hand
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Receptors, Glycine
7.Chlorogenic Acid Improves Neuroprotective Effect of PEP-1-Ribosomal Protein S3 Against Ischemic Insult.
Eun Hee AHN ; Dae Won KIM ; Min Jea SHIN ; Soon Won KWON ; Young Nam KIM ; Duk Soo KIM ; Soon Sung LIM ; Joon KIM ; Jinseu PARK ; Won Sik EUM ; Hyun Sook HWANG ; Soo Young CHOI
Experimental Neurobiology 2011;20(4):169-175
Chlorogenic acid (CGA) possesses various biological activities such as anti-oxidant, anti-inflammatory, and anti-diabetic activities. In the present study, we examined the effect of CGA on the transduction efficiency of PEP-1-ribosomal protein S3 (PEP-1-rpS3) into cells and brain tissues, and its neuroprotective potential against ischemia/reperfusion. We found that, in the presence of CGA, the transduction efficiency of PEP-1-rpS3 into astrocytes and the CA1 region of the hippocampus was enhanced, compared to its transduction in the absence of CGA. Also, cell viability data demonstrated that the sample treated with CGA + PEP-1-rpS3 exhibited improved cell viability against hydrogen peroxide (H2O2)-induced toxicity more significantly than the sample treated with PEP-1-rpS3 alone. Also, in a gerbil ischemia model, data demonstrated that following the ischemic insult, the group treated with PEP-1-rpS3 + CGA showed markedly enhanced protection of neuron cells in CA1 region of hippocampus, compared to those treated with CGA or PEP-1-rpS3 alone. Taken together, these results suggest that CGA may improve the transduction efficiency of protein transduction domain (PTD) fusion proteins into target cells or tissues, thereby enhancing their therapeutic potential against various diseases.
Astrocytes
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Brain
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Cell Survival
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Chlorogenic Acid
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Gerbillinae
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Hippocampus
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Hydrogen Peroxide
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Ischemia
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Neurons
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Neuroprotective Agents
;
Proteins
8.The Serotonin-6 Receptor as a Novel Therapeutic Target.
Experimental Neurobiology 2011;20(4):159-168
Serotonin (5-hydroxytryptamine, 5-HT) is an important neurotransmitter that is found in both the central and peripheral nervous systems. 5-HT mediates its diverse physiological responses through 7 different 5-HT receptor families: 5-HT1, 5-HT2, 5-HT3, 5-HT4, 5-HT5, 5-HT6, and 5-HT7 receptors. Among them, the 5-HT6 receptor (5-HT6R) is the most recently cloned serotonin receptor and plays important roles in the central nervous system (CNS) and in the etiology of neurological diseases. Compared to other 5-HT receptors, the 5-HT6R has been considered as an attractive CNS therapeutic target because it is expressed exclusively in the CNS and has no known isoforms. This review evaluates in detail the role of the 5-HT6R in the physiology and pathophysiology of the CNS and the potential usefulness of 5-HT6R ligands in the development of therapeutic strategies for the treatment of CNS disorders. Preclinical studies provide support for the use of 5-HT6R ligands as promising medications to treat the cognitive dysfunction associated with Alzheimer's disease, obesity, depression, and anxiety.
Alzheimer Disease
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Anxiety
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Central Nervous System
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Clone Cells
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Depression
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Ethylamines
;
Indoles
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Ligands
;
Neurotransmitter Agents
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Obesity
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Peripheral Nervous System
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Protein Isoforms
;
Receptors, Serotonin
;
Serotonin
9.ZigBee-based Wireless Neuro-Stimulator for Improving Stroke Recovery.
Gookhwa KIM ; Hyojeong YUN ; Munho RYU ; Yongil SHIN ; Hyoungihl KIM ; Yoonseok YANG
Experimental Neurobiology 2010;19(3):165-172
Stroke is a leading cause of adult disability and the second-leading cause of death in Korea. It is also the third-leading cause of death in the United States, leading to a serious demand for new interventions to improve the quality of life in stroke survivors. To this end, direct cortical stimulation using an epidural electrode has been reported with promising results in animal and human studies, showing the potential for enhancing the recovery in chronic stroke patients. For optimal results, doctors must be able to modify the stimulation pattern as frequently as needed over a period of time for a given patient. However, severe aftereffects caused by stroke limit patients' activities, making regular doctor visits for treatment difficult. This study aims to develop a prototype of a telemedicine system to enhance stroke recovery by using a ZigBee-based wireless neuro-stimulator. The ZigBee is a stable platform for many low-power wireless applications. To allow stroke patients to remotely obtain neuro-stimulation treatments from their doctors, we connected the ZigBee to the internet. The system also allows doctors to personalize treatment based on the history of the stimulation parameters. The system developed here can also be beneficial as a common platform for a wide range of brain diseases and clinical care for which electric stimulation is used.
Adult
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Animals
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Brain Diseases
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Cause of Death
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Electric Stimulation
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Electrodes
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Humans
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Internet
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Korea
;
Quality of Life
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Stroke
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Survivors
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Telemedicine
;
United States
10.Selective Susceptibility of Human Dopaminergic Neural Stem Cells to Dopamine-Induced Apoptosis.
Sung Man JEON ; Sang Myung CHEON ; Hye Rahn BAE ; Jae Woo KIM ; Seung U KIM
Experimental Neurobiology 2010;19(3):155-164
Dysfunctions of ubiquitin-proteasome system and toxicity of dopamine have been known as the key mechanisms in the pathogenesis of Parkinson's disease (PD) and proteasome inhibitors are widely used in experimental models of PD to reproduce cell death of dopaminergic neurons. In the present study, immortalized human neural stem cells (HB1.F3, F3) and those transfected with human aromatic acid decarboxylase gene (F3.AADC), were used to investigate the mechanism of selective dopaminergic neuronal cell death mediated by dopamine or proteasome inhibitors. Flow cytometric analysis revealed that F3.AADC was more susceptible to dopamine than parental F3 cell which does not carry dopaminergic phenotype. The dopamine-induced apoptosis was mediated by activation of caspases 3 and 9 and cleavage of PARP. Proteasome inhibitors also induced apoptosis in dose-dependent manner but there was no difference between cell types. Prolonged exposure to subtoxic dose of proteasome inhibitors further enhanced dopamine-induced apoptosis in the F3.AADC, and increased presence of alpha-synuclein and ubiquitin-positive inclusions was noted in the cytoplasm of apoptotic cells by immunocytochemistry. These findings indicate that dopaminergic cells are selectively susceptible to dopamine toxicity and prolonged suppression of proteasome system further enhances selective sensitivity to dopamine toxicity. Chronic subtoxic proteasomal dysfunction of dopaminergic cells might contribute to selective cell death of dopaminergic neurons during the pathogenesis of Parkinson's disease.
alpha-Synuclein
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Apoptosis
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Caspases
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Cell Death
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Cytoplasm
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Dopamine
;
Dopaminergic Neurons
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Humans
;
Immunohistochemistry
;
Models, Theoretical
;
Neural Stem Cells
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Parents
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Parkinson Disease
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Phenotype
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Proteasome Endopeptidase Complex
;
Proteasome Inhibitors