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Hereditary Motor and Sensory Neuropathy Type VI with Bilateral Middle Cerebellar Peduncle Involvement.

Jung Hwan OH ; Han Sang LEE ; Dong Min CHA ; Sa Yoon KANG

Experimental Neurobiology.2014;23(3):266-269. doi:10.5607/en.2014.23.3.266

Charcot-Marie-Tooth disease (CMT) 2A with optic atrophy is referred to as hereditary motor and sensory neuropathy type VI (HMSN VI) and is caused by mitofusin 2 gene (MFN2) mutation. In patients with MFN2 related CMT, central nervous system is known to be also involved and cerebral white matter is mostly involved. We report a patient confirmed as HMSN VI who had isolated bilateral middle cerebellar peduncular lesions in brain MRI.
Brain ; Central Nervous System ; Charcot-Marie-Tooth Disease ; Hereditary Sensory and Motor Neuropathy* ; Humans ; Magnetic Resonance Imaging ; Mitochondria ; Optic Atrophy

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Proposed Motor Scoring System in a Porcine Model of Parkinson's Disease induced by Chronic Subcutaneous Injection of MPTP.

Joon Ho MOON ; Ji Ho KIM ; Hyung Jun IM ; Dong Soo LEE ; Eun Jung PARK ; Kilyoung SONG ; Hyun Ju OH ; Su Bin HYUN ; Sang Chul KANG ; Hyunil KIM ; Hyo Eun MOON ; Hyung Woo PARK ; Hong Jae LEE ; Eun Ji KIM ; Seokjoong KIM ; Byeong Chun LEE ; Sun Ha PAEK

Experimental Neurobiology.2014;23(3):258-265. doi:10.5607/en.2014.23.3.258

Destruction of dopaminergic neurons in the substantia nigra pars compacta (SNpc) is a common pathophysiology of Parkinson's disease (PD). Characteristics of PD patients include bradykinesia, muscle rigidity, tremor at rest and disturbances in balance. For about four decades, PD animal models have been produced by toxin-induced or gene-modified techniques. However, in mice, none of the gene-modified models showed all 4 major criteria of PD. Moreover, distinguishing between PD model pigs and normal pigs has not been well established. Therefore, we planned to produce a pig model for PD by chronic subcutaneous administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP), neurotoxin. Changes in behavioral patterns of pigs were thoroughly evaluated and a new motor scoring system was established for this porcine model that was based on the Unified Parkinson's Disease Rating Scale (UPDRS) in human PD patients. In summary, this motor scoring system could be helpful to analyze the porcine PD model and to confirm the pathology prior to further examinations, such as positron emission tomography-computed tomography (PET-CT), which is expensive, and invasive immunohistochemistry (IHC) of the brain.
1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine* ; Animals ; Brain ; Dopaminergic Neurons ; Electrons ; Humans ; Hypokinesia ; Immunohistochemistry ; Injections, Subcutaneous* ; Mice ; Models, Animal ; Muscle Rigidity ; Parkinson Disease* ; Pathology ; Substantia Nigra ; Swine ; Tremor

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Comparison between Flail Arm Syndrome and Upper Limb Onset Amyotrophic Lateral Sclerosis: Clinical Features and Electromyographic Findings.

Byung Nam YOON ; Seong Hye CHOI ; Joung Ho RHA ; Sa Yoon KANG ; Kwang Woo LEE ; Jung Joon SUNG

Experimental Neurobiology.2014;23(3):253-257. doi:10.5607/en.2014.23.3.253

Flail arm syndrome (FAS), an atypical presentation of amyotrophic lateral sclerosis (ALS), is characterized by progressive, predominantly proximal, weakness of upper limbs, without involvement of the lower limb, bulbar, or respiratory muscles. When encountering a patient who presents with this symptomatic profile, possible diagnoses include upper limb onset ALS (UL-ALS), and FAS. The lack of information regarding FAS may make differential diagnosis between FAS and UL-ALS difficult in clinical settings. The aim of this study was to compare clinical and electromyographic findings from patients diagnosed with FAS with those from patients diagnosed with UL-ALS. To accomplish this, 18 patients with FAS and 56 patients with UL-ALS were examined. Significant differences were observed between the 2 groups pertaining to the rate of fasciculation, patterns of predominantly affected muscles, and the Medical Research Council scale of the weakest muscle. The presence of upper motor neuron signs and lower motor neuron involvement evidenced through electromyography showed no significant between-group differences.
Amyotrophic Lateral Sclerosis* ; Arm* ; Diagnosis ; Diagnosis, Differential ; Electromyography ; Fasciculation ; Humans ; Lower Extremity ; Motor Neuron Disease ; Motor Neurons ; Muscles ; Respiratory Muscles ; Upper Extremity*

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Aucubin Promotes Neurite Outgrowth in Neural Stem Cells and Axonal Regeneration in Sciatic Nerves.

Yong Min KIM ; U Cheol SIM ; Yongsung SHIN ; Yunhee Kim KWON

Experimental Neurobiology.2014;23(3):238-245. doi:10.5607/en.2014.23.3.238

Aucubin is an iridoid glycoside with a wide range of biological activities, including anti-inflammatory, anti-microbial, anti-algesic as well as anti-tumor activities. Recently, it has been shown that aucubin prevents neuronal death in the hippocampal CA1 region in rats with diabetic encephalopathy. In addition, it has protective effects on H2O2-induced apoptosis in PC12 cells. We have shown here that aucubin promotes neuronal differentiation and neurite outgrowth in neural stem cells cultured primarily from the rat embryonic hippocampus. We also investigated whether aucubin facilitates axonal elongation in the injured peripheral nervous system. Aucubin promoted lengthening and thickness of axons and re-myelination at 3 weeks after sciatic nerve injury. These results indicate that administration of aucubin improved nerve regeneration in the rat model of sciatic nerve injury, suggesting that aucubin may be a useful therapeutic compound for the human peripheral nervous system after various nerve injuries.
Animals ; Apoptosis ; Axons* ; CA1 Region, Hippocampal ; Hippocampus ; Humans ; Models, Animal ; Nerve Regeneration ; Neural Stem Cells* ; Neurites* ; Neurons ; PC12 Cells ; Peripheral Nervous System ; Rats ; Regeneration* ; Sciatic Nerve*

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Effect of Acute and Chronic Electroconvulsive Shock on 5-Hydroxytrypamine 6 Receptor Immunoreactivity in Rat Hippocampus.

Hyun Jung KIM ; Seungwoo KANG ; Hyun Ju KIM ; Sun Hye CHOI ; Seungkeun SHIN ; Hyung Ha LEE ; Hyewhon RHIM ; Kyung Ho SHIN

Experimental Neurobiology.2014;23(3):231-237. doi:10.5607/en.2014.23.3.231

Electroconvulsive shock (ECS) induces not only an antidepressant effect but also adverse effects such as amnesia. One potential mechanism underlying both the antidepressant and amnesia effect of ECS may involve the regulation of serotonin (5-hydroxytryptamine) 6 (5-HT6) receptor, but less is known about the effects of acute ECS on the changes in 5-HT6 receptor expression in the hippocampus. In addition, as regulation of 5-HT receptor expression is influenced by the number of ECS treatment and by interval between ECS treatment and sacrifice, it is probable that magnitude and time-dependent changes in 5-HT6 receptor expression could be influenced by repeated ECS exposure. To explore this possibility, we observed and compared the changes of 5-HT6 receptor immunoreactivity (5-HT6 IR) in rat hippocampus at 1, 8, 24, or 72 h after the treatment with either a single ECS (acute ECS) or daily ECS for 10 days (chronic ECS). We found that acute ECS increased 5-HT6 IR in the CA1, CA3, and granule cell layer of hippocampus, reaching peak levels at 8 h and returning to basal levels 72 h later. The magnitude and time-dependent changes in 5-HT6 IR observed after acute ECS were not affected by chronic ECS. These results demonstrate that both acute and chronic ECS transiently increase the 5-HT6 IR in rat hippocampus, and suggest that the magnitude and time-dependent changes in 5-HT6 IR in the hippocampus appear not to be influenced by repeated ECS treatment.
Amnesia ; Animals ; Electroshock* ; Hippocampus* ; Rats* ; Serotonin

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The Combination of Antidepressant Duloxetine with Piracetam in Mice does not Produce Enhancement of Nootropic Activity.

Pravin Popatrao KALE ; Veeranjaneyulu ADDEPALLI ; Amrita SARKAR ; Sonam PATEL ; Jay SAVAI

Experimental Neurobiology.2014;23(3):224-230. doi:10.5607/en.2014.23.3.224

There is a strong association between depression and memory impairment. The present study aims to assess the nootropic activity of duloxetine and piracetam combination. Male Swiss Albino mice were divided randomly into 4 groups. Treatment of normal saline (10 ml/kg), duloxetine (10 mg/kg), piracetam (100 mg/kg), and duloxetine (5 mg/kg) plus piracetam (50 mg/kg) were given through intra-peritoneal route to group I-IV, respectively. Transfer latency in elevated plus maze (EPM) and time spent in target quadrant in Morris water maze (MWM) were recorded. Estimation of brain monoamines in hippocampus, cerebral cortex, and whole brain were done using HPLC with fluorescence detector. Piracetam treated group showed significant decrease in transfer latency in EPM and increase in time spent in target quadrant recorded in MWM. Combination treated group failed to produce statistically significant nootropic effect in both EPM and MWM. Combination treated group failed to increase brain monoamine levels when compared against duloxetine and piracetam treated groups, separately. But there was exception of significant increase in norepinephrine levels in hippocampi when compared against duloxetine treated group. Results indicate no cognitive benefits with piracetam plus duloxetine combination. These findings can be further probed with the aim of understanding the interaction between duloxetine and piracetam as a future endeavor.
Animals ; Brain ; Cerebral Cortex ; Chromatography, High Pressure Liquid ; Depression ; Fluorescence ; Hippocampus ; Humans ; Male ; Maze Learning ; Memory ; Mice* ; Norepinephrine ; Piracetam* ; Duloxetine Hydrochloride

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Finger Motion Decoding Using EMG Signals Corresponding Various Arm Postures.

Kyung Jin YOU ; Ki Won RHEE ; Hyun Chool SHIN

Experimental Neurobiology.2010;19(1):54-61. doi:10.5607/en.2010.19.1.54

We provide a novel method to infer finger flexing motions using a four-channel surface electromyogram (EMG). Surface EMG signals can be recorded from the human body non-invasively and easily. Surface EMG signals in this study were obtained from four channel electrodes placed around the forearm. The motions consist of the flexion of five single fingers (thumb, index finger, middle finger, ring finger, and little finger) and three multi-finger motions. The maximum likelihood estimation was used to infer the finger motions. Experimental results have shown that this method can successfully infer the finger flexing motions. The average accuracy was as high as 97.75%. In addition, we examined the influence of inference accuracies with the various arm postures.
Arm ; Electrodes ; Fingers ; Forearm ; Human Body ; Posture

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Ectopic Expression of Ephrin-A5 Under the EphA8 Promoter at the Anterior Region of the Superior Colliculus.

Hyuna NOH ; Soochul PARK

Experimental Neurobiology.2010;19(1):49-53. doi:10.5607/en.2010.19.1.49

EphA/ephrin-A mediated signaling has emerged as a key mechanism regulating axon guidance and topographic mapping, particularly in the well-characterized visual system from the retina to the superior colliculus (SC). In this study, EphA8 bacterial artificial chromosome (BAC) was manipulated to contain a floxed eGFP and human ephrin-A5 expression cassette using homologous recombination method. In the mice containing the recombinant BAC, it was shown that GFP is expressed in an anterior>posterior gradient in the SC. Furthermore, when these mice were crossed with the transgenic mice expressing Cre under the EphA8 promoter, it was evident that a GFP expression cassette was eliminated, and that human ephrin-A5 was ectopically expressed in the anterior region of the SC. This transgenic model would be useful to analyze the role of ephrin-A5 in the SC during the retinocollicular topography formation.
Animals ; Axons ; Chromosomes, Artificial, Bacterial ; Ephrin-A5 ; Homologous Recombination ; Humans ; Mice ; Mice, Transgenic ; Retina ; Superior Colliculi

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Normal Adult Hippocampal Neurogenesis in SRG3-overexpressing Transgenic Mice.

Byungwoo KIM ; Eugene LEE ; Rho H SEONG ; Heekyoung CHUNG ; Hyeon SON

Experimental Neurobiology.2010;19(1):39-48. doi:10.5607/en.2010.19.1.39

SRG3 (SWI3-related gene) is a core subunit of mouse SWI/SNF complex and is known to play a critical role in stabilizing the SWI/SNF complex by attenuating its proteasomal degradation. SWI/SNF chromatin remodeling complex is reported to act as an important endogenous regulator in the proliferation and differentiation of mammalian neural stem cells. Because limited expression of SRG3 occurs in the brain and thymus during mouse embryogenesis, it was hypothesized that the altered SRG3 expression level might affect the process of adult hippocampal neurogenesis. Due to the embryonic lethality of homozygous knockout mice, this study focuses on dissecting the effect of overexpressed SRG3 on adult hippocampal neurogenesis. The BrdU incorporation assay, immunostaing with neuronal markers for each differentiation stage, and imunoblotting analysis with intracellular molecules involved in survival in adult hippocampal neurogenesis found no alteration, suggesting that the overexpression of SRG3 protein in mature neurons had no effect on the entire process of adult hippocampal neurogenesis including proliferation, differentiation, and survival.
Adult ; Animals ; Brain ; Bromodeoxyuridine ; Chromatin Assembly and Disassembly ; Embryonic Development ; Female ; Humans ; Mice ; Mice, Knockout ; Mice, Transgenic ; Neural Stem Cells ; Neurogenesis ; Neurons ; Pregnancy ; Thymus Gland

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Antidepressant-like Effect of Kaempferol and Quercitirin, Isolated from Opuntia ficus-indica var. saboten.

Soo Hyun PARK ; Yun Beom SIM ; Pyung Lim HAN ; Jin Koo LEE ; Hong Won SUH

Experimental Neurobiology.2010;19(1):30-38. doi:10.5607/en.2010.19.1.30

Opuntia ficus-indica var. saboten. is widely cultivated in Jeju Island (South Korea) for use in manufacture of health foods. This study described antidepressant effect of two flavonoids (kaempferol and quercitrin) isolated from the Opuntia ficus-indica var. saboten. The expression of the hypothalamic POMC mRNA or plasma beta-endorphin levels were increased by extract of Opintia ficus-indica var. saboten or its flavoniods administered orally. In addition, antidepressant activity was studied using tail suspension test (TST), forced swimming test (FST) and rota-rod test in chronically restraint immobilization stress group in mice. After restraint stress (2 hrs/day for 14 days), animals were kept in cage for 14 days without any further stress, bet with drugs. Mice were fed with a diet supplemented for 14 days and during the behavioral test period with kaempferol or quercitrin (30 mg/kg/day). POMC mRNA or plasma beta-endorphin level was increased by extract of Opintia ficus-indica var. saboten and its flavoniods. In addition, immobility time in TST and FST was significantly reduced by kaempferol or quercitrin. In rota-rod test, the time of permanence was maintained to the semblance of control group in turning at 15 rpm. Our results suggest that two flavonoids (kaempferol and quercitrin) isolated from the Opuntia ficus-indica var. saboten. show a potent antidepressant effect.
Animals ; beta-Endorphin ; Diet ; Flavonoids ; Hindlimb Suspension ; Immobilization ; Kaempferols ; Mice ; Opuntia ; Plasma ; Pro-Opiomelanocortin ; Quercetin ; RNA, Messenger ; Swimming ; Food, Organic

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