• BMB Reports
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• v.55 no.7
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• pp.323-335
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• 2022

Together with neuronal loss, the existence of insoluble inclusions of alpha-synuclein (α-syn) in the brain is widely accepted as a hallmark of synucleinopathies including Parkinson's disease (PD), multiple system atrophy, and dementia with Lewy body. Because the α-syn aggregates are deeply involved in the pathogenesis, there have been many attempts to demonstrate the mechanism of the aggregation and its potential causative factors including post-translational modifications (PTMs). Although no concrete conclusions have been made based on the previous study results, growing evidence suggests that modifications such as phosphorylation and ubiquitination can alter α-syn characteristics to have certain effects on the aggregation process in PD; either facilitating or inhibiting fibrillization. In the present work, we reviewed studies showing the significant impacts of PTMs on α-syn aggregation. Furthermore, the PTMs modulating α-syn aggregation-induced cell death have been discussed.

• BMB Reports
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• v.55 no.5
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• pp.238-243
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• 2022

Autism or autism spectrum disorder (ASD) is a behavioral syndrome characterized by persistent deficits in social interaction, and repetitive patterns of behavior, interests, or activities. The gene encoding Methyl-CpG binding protein 2 (MeCP2) is one of a few exceptional genes of established causal effect in ASD. Although genetically engineered mice studies may shed light on how MeCP2 loss affects synaptic activity patterns across the whole brain, such studies are not considered practical in ASD patients due to the overall level of impairment, and are technically challenging in mice. For the first time, we show that hippocampal MeCP2 knockdown produces behavioral abnormalities associated with autism-like traits in rats, providing a new strategy to investigate the efficacy of therapeutics in ASD. Ketamine, an N-Methyl-D-aspartate (NMDA) blocker, has been proposed as a possible treatment for autism. Using the MeCP2 knockdown rats in conjunction with a rat model of valproic acid (VPA)-induced ASD, we examined gene expression and ASD behaviors upon ketamine treatment. We report that the core symptoms of autism in MeCP2 knockdown rats with social impairment recovered dramatically following a single treatment with ketamine.

• Journal of Korean Academy of Nursing
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• v.45 no.2
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• pp.202-210
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• 2015

Purpose: The purpose of this study was to describe depression, caregiving burden and the correlation of the two variables in the families of patients with amyotrophic lateral sclerosis (ALS) and to clarify factors predicting caregiving burden. Methods: A descriptive and cross-sectional study was conducted with 139 family members who provided care to patients with ALS. The characteristics of patients and families, Korean-Beck Depression Inventory (K-BDI), Korean version of Zarit Burden Interview (K-ZBI) and Korean-Amyotrophic Lateral Sclerosis Functional Rating Scale - Revised (K-ALSFRS-R) were used as study measures. Results: The mean score for K-BDI was 19.39 out of 63 suggesting sub-clinical depression and 38.2% of the family members exhibited depression. The mean score for K-ZBI was 66.03 out of 88. The predictors for K-ZBI were K-BDI, age of family member, length of time spent per day in caring, relationship to patient and K-ALSFRS-R. Conclusion: The results of this study suggest that levels of depression and caregiving burden are high among family members caring for patients with ALS. As depression is associated with caregiving burden, screening and emotional supports should be provided to reduce the burden of care for these family. Support programs to alleviate the care burden are also needed, considering family demographics, time per day in caring giving and K-ALSFRS-R.

• Tuberculosis and Respiratory Diseases
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• v.71 no.6
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• pp.464-469
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• 2011

Adalimumab is a full human monoclonal antibody that inhibits tumor necrosis factor-alpha (TNF-${\alpha}$). This has recently been shown to be effective in the treatment of rheumatoid arthritis (RA), ankylosing spondylitis, and other conditions. Sacoidosis is known to be the target for adalimumab but we describe a patient who has developed sarcoidosis with lung involvement during adalimumab therapy for RA. A 48-year-old woman, who was treated with adalimumab for 5 months, was admitted because of chronic cough and both hilar lymphadenopathy on chest radiography. Chest computed tomography revealed the enlargement of multiple lymph nodes in the right supraclavicular, subcarinal, both hilar and right axillary area. She was diagnosed with sarcoidosis based on the biopsy of supraclavicular lymph node, skin and lung through video-associated thoracoscopic surgery, which was non-caseating epitheloid cell granuloma and excluded from a similar disease. She was treated for sarcoidosis with prednisolone and methotrexate instead of adalimumab.

• Tuberculosis and Respiratory Diseases
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• v.39 no.2
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• pp.186-193
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• 1992

Eosinophilic granuloma (histiocytosis $\times$) is an uncommon granulomatous disorder affecting the mononuclear phagocytic cell system caused by unknown etiology. When confined to the lungs, it is also known as pulmonary eosinophilic granuloma or pulmonary histiocytosis $\times$. After the first case was described in 1951 by Farinacci et ai, more 250 cases were reported in the English literature. However only 4 cases has been introduced in our country. We describe two additional cases. In both cases, open lung biopsies are performed for definite diagnosis and examined by immunohistochemical stain for S-100 protein and electron microscopy for ultrastructural identification of Birbeck granule.

• Journal of Chest Surgery
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• v.39 no.10 s.267
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• pp.795-798
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• 2006

Thymic carcinoid tumor associated with Cushing's syndrome is a rare disease with a poor prognosis. Thymic carcinoid with Cushing's syndrome caused by CRH (corticotropin-releasing hormone) production is even rarer. We report a 58-year-old woman with a huge anterior mediastinal mass. Five months after thymectomy the patient was readmitted with symptoms of generalized edema and dyspnea. Recurrence and metastases were discovered and Cushing's syndrome diagnosed.

• Journal of Chest Surgery
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• v.34 no.6
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• pp.506-510
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• 2001

Langerhans\` Cell Histiocytosis(LCH) is a disease of unknown origin, characterized by abnormal proliferation of Langerhans\` cells. Previously, it has been called histiocytosis X, which included eosinophilic granuloma, Hand-Sch ller-Christian\`s disease, and Letterer-Siwe disease. Any organs or tissues such as skin, lymph nodes, bone and bone marrow can be involved. However, LCH of chest wall is rarely reported in our country. We experienced a 18 month old male child, who had osteolytic lesion involving the rib with axillary lymph node metastasis. The tumor was confirmed as LCH after surgery.

• Archives of Craniofacial Surgery
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• v.20 no.1
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• pp.37-43
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• 2019

Background: Tongue reconstruction is challenging with the unique function and anatomy. Goals for reconstruction differ depending on the extent of reconstruction. Thin and pliable flaps are useful for tongue tip reconstruction, for appearance and mobility. This study reports lateral arm free flap (LAFF) as a safe and optimal option for hemi-tongue reconstruction, especially for tongue tip after hemiglossectomy. Methods: Thirteen LAFFs were performed for hemi-tongue reconstruction after hemiglossectomy from 1995 to 2018. Of the 13 patients, seven were male and six were female, age varying from 24 to 64 years. Results: All flaps healed uneventfully without complications. Donor sites were closed primarily. The recipient vessels for microvascular anastomosis were mainly superior thyroidal artery, external jugular vein. All patients returned to normal diet, with no complaints regarding reconstructed tongue and donor site. Conclusion: The LAFF is hairless, thin (especially with lateral epicondyle approach), and potentially sensate. They are advantageous features for tongue tip and hemi-tongue reconstruction. Donor site sacrifices the inessential posterior radial collateral artery, and the scar is hidden under short sleeve shirts. We believe that LAFF can be considered as the first choice flap for hemitongue reconstruction, over radial forearm free flaps.

• BMB Reports
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• v.52 no.6
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• pp.391-396
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• 2019

Receptor activator of nuclear factor kappa B ligand (RANKL) expression in osteoblasts is regulated by 1,25-dihydroxyvitamin D3 (1,25D3). CCAAT/enhancer-binding protein beta ($C/EBP{\beta}$) has been proposed to function as a transcription factor and upregulate RANKL expression, but it is still uncertain how $C/EBP{\beta}$ is involved in 1,25D3-induced RANKL expression of osteoblasts. 1,25D3 stimulation increased the expression of RANKL and $C/EBP{\beta}$ genes in osteoblasts and enhanced phosphorylation and stability of these proteins. Moreover, induction of RANKL expression by 1,25D3 in osteoblasts was downregulated upon knockdown of $C/EBP{\beta}$. In contrast, $C/EBP{\beta}$ overexpression directly upregulated RANKL promoter activity and exhibited a synergistic effect on 1,25D3-induced RANKL expression. In particular, 1,25D3 treatment of osteoblasts increased $C/EBP{\beta}$ protein binding to the RANKL promoter. In conclusion, $C/EBP{\beta}$ is required for induction of RANKL by 1,25D3.

• Proceedings of the Korean Magnestics Society Conference
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• 2006.11a
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• pp.56-56
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• 2006