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Your search keyword '"Ki, Chang Seok"' showing total 859 results
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859 results on '"Ki, Chang Seok"'

5. Integrative modeling of tumor genomes and epigenomes for enhanced cancer diagnosis by cell-free DNA

Author

Bae, Mingyun, Kim, Gyuhee, Lee, Tae-Rim, Ahn, Jin Mo, Park, Hyunwook, Park, Sook Ryun, Song, Ki Byung, Jun, Eunsung, Oh, Dongryul, Lee, Jeong-Won, Park, Young Sik, Song, Ki-Won, Byeon, Jeong-Sik, Kim, Bo Hyun, Sohn, Joo Hyuk, Kim, Min Hwan, Kim, Gun Min, Chie, Eui Kyu, Kang, Hyun-Cheol, Kong, Sun-Young, Woo, Sang Myung, Lee, Jeong Eon, Ryu, Jai Min, Lee, Junnam, Kim, Dasom, Ki, Chang-Seok, Cho, Eun-Hae, and Choi, Jung Kyoon

Published
2023
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17. Directly converted patient-specific induced neurons mirror the neuropathology of FUS with disrupted nuclear localization in amyotrophic lateral sclerosis

Author

Lim, Su Min, Choi, Won Jun, Oh, Ki-Wook, Xue, Yuanchao, Choi, Ji Young, Kim, Sung Hoon, Nahm, Minyeop, Kim, Young-Eun, Lee, Jinhyuk, Noh, Min-Young, Lee, Seungbok, Hwang, Sejin, Ki, Chang-Seok, Fu, Xiang-Dong, and Kim, Seung Hyun

Subjects
Biochemistry and Cell Biology, Biomedical and Clinical Sciences, Biological Sciences, Neurodegenerative, Stem Cell Research - Induced Pluripotent Stem Cell, Stem Cell Research - Induced Pluripotent Stem Cell - Human, Brain Disorders, Rare Diseases, Neurosciences, Genetics, Stem Cell Research, ALS, Aetiology, 2.1 Biological and endogenous factors, Neurological, Adult, Amyotrophic Lateral Sclerosis, Cell Nucleus, Cytoplasmic Granules, Female, Humans, Inclusion Bodies, Male, Motor Neurons, Mutant Proteins, Mutation, Neuropathology, RNA-Binding Protein FUS, Amyotrophic lateral sclerosis, Fused in sarcoma, Human cell models, Induced neuron, Nuclear localization signal, Stress granules, Neuronal cytoplasmic inclusion, Clinical Sciences, Neurology & Neurosurgery, Biochemistry and cell biology
Abstract

BackgroundMutations in the fused in sarcoma (FUS) gene have been linked to amyotrophic lateral sclerosis (ALS). ALS patients with FUS mutations exhibit neuronal cytoplasmic mislocalization of the mutant FUS protein. ALS patients' fibroblasts or induced pluripotent stem cell (iPSC)-derived neurons have been developed as models for understanding ALS-associated FUS (ALS-FUS) pathology; however, pathological neuronal signatures are not sufficiently present in the fibroblasts of patients, whereas the generation of iPSC-derived neurons from ALS patients requires relatively intricate procedures.ResultsHere, we report the generation of disease-specific induced neurons (iNeurons) from the fibroblasts of patients who carry three different FUS mutations that were recently identified by direct sequencing and multi-gene panel analysis. The mutations are located at the C-terminal nuclear localization signal (NLS) region of the protein (p.G504Wfs*12, p.R495*, p.Q519E): two de novo mutations in sporadic ALS and one in familial ALS case. Aberrant cytoplasmic mislocalization with nuclear clearance was detected in all patient-derived iNeurons, and oxidative stress further induced the accumulation of cytoplasmic FUS in cytoplasmic granules, thereby recapitulating neuronal pathological features identified in mutant FUS (p.G504Wfs*12)-autopsied ALS patient. Importantly, such FUS pathological hallmarks of the patient with the p.Q519E mutation were only detected in patient-derived iNeurons, which contrasts to predominant FUS (p.Q519E) in the nucleus of both the transfected cells and patient-derived fibroblasts.ConclusionsThus, iNeurons may provide a more reliable model for investigating FUS mutations with disrupted NLS for understanding FUS-associated proteinopathies in ALS.

Published
2016

18. Patient fibroblasts-derived induced neurons demonstrate autonomous neuronal defects in adult-onset Krabbe disease

Author

Lim, Su Min, Choi, Byung-Ok, Oh, Seong-il, Choi, Won Jun, Oh, Ki-Wook, Nahm, Minyeop, Xue, Yuanchao, Choi, Jae Hyeok, Choi, Ji Young, Kim, Young-Eun, Chung, Ki Wha, Fu, Xiang-Dong, Ki, Chang-Seok, and Kim, Seung Hyun

Subjects
Neurosciences, Neurodegenerative, Stem Cell Research, Stem Cell Research - Nonembryonic - Human, Brain Disorders, 2.1 Biological and endogenous factors, Aetiology, Neurological, Animals, Brain, Case-Control Studies, Cell Transdifferentiation, Child, DNA Mutational Analysis, Disease Models, Animal, Enzyme Activation, Female, Fibroblasts, Galactosylceramidase, Genetic Association Studies, Humans, Leukodystrophy, Globoid Cell, Lysosomes, Magnetic Resonance Imaging, Male, Mutation, Neurons, Pedigree, Protein Transport, Psychosine, krabbe disease, globoid cell leukodystrophy, beta-galactosylceramidase, psychosine, induced neuron, Gerotarget, β-galactosylceramidase, Krabbe disease, Oncology and Carcinogenesis
Abstract

Krabbe disease (KD) is an autosomal recessive neurodegenerative disorder caused by defective β-galactosylceramidase (GALC), a lysosomal enzyme responsible for cleavage of several key substrates including psychosine. Accumulation of psychosine to the cytotoxic levels in KD patients is thought to cause dysfunctions in myelinating glial cells based on a comprehensive study of demyelination in KD. However, recent evidence suggests myelin-independent neuronal death in the murine model of KD, thus indicating defective GALC in neurons as an autonomous mechanism for neuronal cell death in KD. These observations prompted us to generate induced neurons (iNeurons) from two adult-onset KD patients carrying compound heterozygous mutations (p.[K563*];[L634S]) and (p.[N228_S232delinsTP];[G286D]) to determine the direct contribution of autonomous neuronal toxicity to KD. Here we report that directly converted KD iNeurons showed not only diminished GALC activity and increased psychosine levels, as expected, but also neurite fragmentation and abnormal neuritic branching. The lysosomal-associated membrane proteins 1 (LAMP1) was expressed at higher levels than controls, LAMP1-positive vesicles were significantly enlarged and fragmented, and mitochondrial morphology and its function were altered in KD iNeurons. Strikingly, we demonstrated that psychosine was sufficient to induce neurite defects, mitochondrial fragmentation, and lysosomal alterations in iNeurons derived in healthy individuals, thus establishing the causal effect of the cytotoxic GALC substrate in KD and the autonomous neuronal toxicity in KD pathology.

Published
2016

26. Proteolytically degradable PEG hydrogel matrix mimicking tumor immune microenvironment for 3D co-culture of lung adenocarcinoma cells and macrophages

Author

Lee, Sora and Ki, Chang Seok

Subjects
Biomaterials, Biomedical Engineering, Biophysics, Bioengineering
Abstract

Tumor-associated macrophages and monocytes are the major stromal cell types found in the tumor immune microenvironment (TIME), which modulates tumor progression, invasion, and chemoresistance. To address the need for an in vitro three-dimensional tumor model for understanding the complex cellular interactions within the TIME, we propose a TIME-mimetic co-culture matrix composed of photo-crosslinked poly(ethylene glycol) hydrogels mimicking the characteristics of the tumor and stroma. Desmoplasia-mimetic microgels encapsulating lung adenocarcinoma cells (A549) were embedded with monocyte- or macrophage-type U937 cells in normal stroma-mimetic hydrogel, increasing the proximity between the two cell types. By modulating the proteolytic degradability of the hydrogels, we could separate different cell types with high purities for use in orthogonal assays. In addition, we demonstrated that U937 cells had distinct influences on A549 cell death depending on their activation states (i.e. monocyte, M0, or M1 phenotype). M1 macrophages suppressed tumor growth and increased the susceptibility of A549 cells to cisplatin. In contrast, monocytes upregulated cancer stem cell markers (OCT4, SOX2, and SHH) of A549 cells, showing M2-like features, such as downregulated expression of proinflammatory markers (IL6 and TNFα). These findings suggest that this co-culture system is potentially used for investigation of heterotypic cellular interactions in the TIME. The developed co-culture model successfully reproduces complex tumor immune microenvironment.The model is composed of tumor- and stroma-mimetic matrices containing tumor and immune cells.Sequential matrix degradation enabled the independent cell collection. The developed co-culture model successfully reproduces complex tumor immune microenvironment. The model is composed of tumor- and stroma-mimetic matrices containing tumor and immune cells. Sequential matrix degradation enabled the independent cell collection.

Published
2023

39. Abstract 6697: Deep learning algorithm for cancer detection using multimodal characteristics of whole methylome sequencing of cf-DNA

Author

Park, Juntae, primary, Kim, Minjung, additional, Park, Sook Ryun, additional, Song, Ki-Byung, additional, Jun, Eunsung, additional, Oh, Dongryul, additional, Lee, Jeong-Won, additional, Park, Young Sik, additional, Song, Ki-Won, additional, Byeon, Jeong-Sik, additional, Kim, Bo Hyun, additional, Ki, Chang-Seok, additional, and Cho, Eunhae, additional

Published
2023
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44. Author Correction: Multimodal imaging analyses in patients with genetic and sporadic forms of small vessel disease

Author

Kim, Ko Woon, Kwon, Hunki, Kim, Young-Eun, Yoon, Cindy W., Kim, Yeo Jin, Kim, Yong Bum, Lee, Jong Min, Yoon, Won Tae, Kim, Hee Jin, Lee, Jin San, Jang, Young Kyoung, Kim, Yeshin, Jang, Hyemin, Ki, Chang-Seok, Youn, Young Chul, Shin, Byoung-Soo, Bang, Oh Young, Kim, Gyeong-Moon, Chung, Chin-Sang, Kim, Seung Joo, Na, Duk L., Duering, Marco, Cho, Hanna, and Seo, Sang Won

Published
2019
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