• Title/Summary/Keyword: Brain Stem

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Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia

  • Lee, Yun-Jin;Chung, Kee-Yang;Kang, Hoon-Chul;Kim, Heung Dong;Lee, Joon Soo
    • Clinical and Experimental Pediatrics
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    • v.58 no.9
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    • pp.354-357
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    • 2015
  • Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied. Brain magnetic resonance imaging revealed hemiatrophy of the face and skull base, and T2-weighted images showed increased signal in the right hemipons and hemicerebellar peduncle. Magnetic resonance angiography findings were unremarkable. She was treated with oral prednisolone, and her recurrent gait ataxia diminished within 2 months of the follow-up period. To the best of our knowledge, this is only the second case of PRS presenting with an abnormal involvement of the ipsilateral hemipons.

Medulloblastoma in the Molecular Era

  • Kuzan-Fischer, Claudia Miranda;Juraschka, Kyle;Taylor, Michael D.
    • Journal of Korean Neurosurgical Society
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    • v.61 no.3
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    • pp.292-301
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    • 2018
  • Medulloblastoma is the most common malignant brain tumor of childhood and remains a major cause of cancer related mortality in children. Significant scientific advancements have transformed the understanding of medulloblastoma, leading to the recognition of four distinct clinical and molecular subgroups, namely wingless (WNT), sonic hedgehog, group 3, and group 4. Subgroup classification combined with the recognition of subgroup specific molecular alterations has also led to major changes in risk stratification of medulloblastoma patients and these changes have begun to alter clinical trial design, in which the newly recognized subgroups are being incorporated as individualized treatment arms. Despite these recent advancements, identification of effective targeted therapies remains a challenge for several reasons. First, significant molecular heterogeneity exists within the four subgroups, meaning this classification system alone may not be sufficient to predict response to a particular therapy. Second, the majority of novel agents are currently tested at the time of recurrence, after which significant selective pressures have been exerted by radiation and chemotherapy. Recent studies demonstrate selection of tumor sub-clones that exhibit genetic divergence from the primary tumor, exist within metastatic and recurrent tumor populations. Therefore, tumor resampling at the time of recurrence may become necessary to accurately select patients for personalized therapy.

Identification of Differentially Expressed Genes in Human Mesenchymal Stem Cell-Derived Neurons

  • Heo, Ji-Hye;Cho, Kyung-Jin;Choi, Dal-Woong;Kim, Suhng-Wook
    • Toxicological Research
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    • v.26 no.1
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    • pp.15-19
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    • 2010
  • Mesenchymal stem cells (MSCs) have greater potential for immediate clinical and toxicological applications, due to their ability to self-renew, proliferate, and differentiate into a variety of cell types. To identify novel candidate genes that were specifically expressed during transdifferentiation of human MSCs to neuronal cells, we performed a differential expression analysis with random priming approach using annealing control primer-based differential display reverse transcription-polymerase chain reaction approach. We identified genes for acyl-CoA thioesterase, tissue inhibitor of metalloproteinases-1, brain glycogen phosphorylase, ubiquitin C-terminal hydrolase and aldehyde reductase were up-regualted, whereas genes for transgelin and heparan sulfate proteoglycan were down-regulated in MSC-derived neurons. These differentially expressed genes may have potential role in regulation of neurogenesis. This study could be applied to environmental toxicology in the field of testing the toxicity of a chemical or a physical agent.

Force-mediated proinvasive matrix remodeling driven by tumor-associated mesenchymal stem-like cells in glioblastoma

  • Lim, Eun-Jung;Suh, Yongjoon;Kim, Seungmo;Kang, Seok-Gu;Lee, Su-Jae
    • BMB Reports
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    • v.51 no.4
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    • pp.182-187
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    • 2018
  • In carcinoma, cancer-associated fibroblasts participate in force-mediated extracellular matrix (ECM) remodeling, consequently leading to invasion of cancer cells. Likewise, the ECM remodeling actively occurs in glioblastoma (GBM) and the consequent microenvironmental stiffness is strongly linked to migration behavior of GBM cells. However, in GBM the stromal cells responsible for force-mediated ECM remodeling remain unidentified. We show that tumor-associated mesenchymal stem-like cells (tMSLCs) provide a proinvasive matrix condition in GBM by force-mediated ECM remodeling. Importantly, CCL2-mediated Janus kinase 1 (JAK1) activation increased phosphorylation of myosin light chain 2 in tMSLCs and led to collagen assembly and actomyosin contractility. Collectively, our findings implicate tMSLCs as stromal cells providing force-mediated proinvasive ECM remodeling in the GBM microenvironment, and reminiscent of fibroblasts in carcinoma.

Effect of Combination Electroacupuncture and Tenuigenin on the Migration and Differentiation of Mesenchymal Stem Cells following Ischemic Stroke

  • Jae Ho Lee;Byung Tae Choi;Hwa Kyoung Shin
    • Journal of Pharmacopuncture
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    • v.26 no.4
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    • pp.357-365
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    • 2023
  • Objectives: Since stroke is a serious health issue, novel therapeutic strategies are required. In a mouse model of ischemic stroke, this study analyzed the potential of electroacupuncture (EA) and tenuigenin (TE) to improve the efficacy of human mesenchymal stem cell (hMSC) transplantation. Methods: Middle cerebral artery occlusion (MCAO) with reperfusion was used to generate ischemic stroke. Forty-eight male C57BL/6 mice were randomly divided into five groups: control, MCAO-operated, MCAO-EA, MCAO-TE, or MCAO + EA + TE. Subsequently, hMSCs were transplanted into the ischemic region and EA, TE, or the combination was administered. Behavior assessments and immunohistochemistry were conducted to evaluate motor and cognitive recovery and hMSCs survival, migration, and differentiation. Results: The combined treatment of EA and TE exhibited enhanced hMSCs survival, migration and differentiation into neural cell lineages while suppressing astrocyte formation. Immunohistochemistry demonstrated increased neurogenesis through hMSCs transplantation in the ischemic brain. Immediate behavioral improvements were not significantly different between groups, but there was a gradual recovery in motor and cognitive function over time. Conclusion: These findings highlight the potential of EA and TE co-treatment as a therapeutic strategy for ischemic stroke, opening avenues for further research to optimize treatment protocols and elucidate underlying mechanisms.

Bortezomib Is Toxic but Induces Neurogenesis and Inhibits TUBB3 Degradation in Rat Neural Stem Cells

  • Seung Yeon Sohn;Thin Thin San;Junhyung Kim;Hyun-Jung Kim
    • Biomolecules & Therapeutics
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    • v.32 no.1
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    • pp.65-76
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    • 2024
  • Bortezomib (BTZ) is a proteasome inhibitor used to treat multiple myeloma (MM). However, the induction of peripheral neuropathy is one of the major concerns in using BTZ to treat MM. In the current study, we have explored the effects of BTZ (0.01-5 nM) on rat neural stem cells (NSCs). BTZ (5 nM) induced cell death; however, the percentage of neurons was increased in the presence of mitogens. BTZ reduced the B-cell lymphoma 2 (Bcl-2)/Bcl-2 associated X protein ratio in proliferating NSCs and differentiated cells. Inhibition of βIII-tubulin (TUBB3) degradation was observed, but not inhibition of glial fibrillary acidic protein degradation, and a potential PEST sequence was solely found in TUBB3. In the presence of growth factors, BTZ increased cAMP response element-binding protein (CREB) phosphorylation, brain-derived neurotrophic factor (Bdnf) transcription, BDNF expression, and Tubb3 transcription in NSCs. However, in the neuroblastoma cell line, SH-SY5Y, BTZ (1-20 nM) only increased cell death without increasing CREB phosphorylation, Bdnf transcription, or TUBB3 induction. These results suggest that although BTZ induces cell death, it activates neurogenic signals and induces neurogenesis in NSCs.

Kaposi's Sarcoma-Associated Herpesvirus Infection Modulates the Proliferation of Glioma Stem-Like Cells

  • Jeon, Hyungtaek;Kang, Yun Hee;Yoo, Seung-Min;Park, Myung-Jin;Park, Jong Bae;Lee, Seung-Hoon;Lee, Myung-Shin
    • Journal of Microbiology and Biotechnology
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    • v.28 no.1
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    • pp.165-174
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    • 2018
  • Glioblastoma multiforme is the most lethal malignant brain tumor. Despite many intensive studies, the prognosis of glioblastoma multiforme is currently very poor, with a median overall survival duration of 14 months and 2-year survival rates of less than 10%. Although viral infections have been emphasized as potential cofactors, their influences on pathways that support glioblastoma progression are not known. Some previous studies indicated that human Kaposi's sarcoma-associated herpesvirus (KSHV) was detected in healthy brains, and its microRNA was also detected in glioblastoma patients' plasma. However, a direct link between KSHV infection and glioblastoma is currently not known. In this study, we infected glioblastoma cells and glioma stem-like cells (GSCs) with KSHV to establish an in vitro cell model for KSHV-infected glioblastoma cells and glioma stem-like cells in order to identify virologic outcomes that overlap with markers of aggressive disease. Latently KSHV-infected glioblastoma cells and GSCs were successfully established. Additionally, using these cell models, we found that KSHV infection modulates the proliferation of glioma stem-like cells.

Isolation of Mesenchymal Stem-like Cells from a Pituitary Adenoma Specimen

  • Shim, Jin-Kyoung;Kang, Seok-Gu;Lee, Ji-Hyun;Chang, Jong Hee;Hong, Yong-Kil
    • Biomedical Science Letters
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    • v.19 no.4
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    • pp.295-302
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    • 2013
  • Some of the pituitary adenomas are invasive and spread into neighboring tissues. In previous studies, the invasion of pituitary adenomas is thought to be associated with epithelial-mesenchymal transition (EMT). In addition to that, we thought that mesenchymal stem cells (MSCs) exist in relevant microenvironment in pituitary adenoma. However, it has been little known about the existence of MSCs from pituitary adenoma. So we investigated whether mesenchymal stem-like cells (MSLCs) can be isolated from the pituitary adenoma specimen. We isolated and cultured candidate MSLCs from the fresh pituitary adenoma specimen with the same protocols used in culturing bone marrow derived MSCs (BM-MSCs). The cultured candidate MSLCs were analyzed by fluorescence-activated cell sorting (FACS) for surface markers associated with MSCs. Candidate MSLCs were exposed to mesenchymal differentiation conditions to determine the mesenchymal differentiation potential of these cells. To evaluate the tumorigenesis of candidate MSLCs from pituitary adenoma, we implanted these cells into the brain of athymic nude mice. We isolated cells resembling BM-MSCs named pituitary adenoma stroma mesenchymal stem-like cells (PAS-MSLCs). PAS-MSLCs were spindle shaped and had adherent characteristics. FACS analysis identified that the PAS-MSLCs had a bit similar surface markers to BM-MSCs. Isolated cells expressed surface antigen, positive for CD105, CD75, and negative for CD45, NG2, and CD90. We found that these cells were capable of differentiation into adipocytes, osteocytes and chondrocytes. Tumor was not developed in the nude mice brains that were implanted with the PAS-MSLCs. In this study, we showed that MSLCs can be isolated from a pituitary adenoma specimen which is not tumorigenic.