A5047391677- Amyotrophic Lateral Sclerosis Research
- Neurogenetic and Muscular Disorders Research
- Parkinson's Disease Mechanisms and Treatments
- Neurological diseases and metabolism
- Mesenchymal stem cell research
- Neuroinflammation and Neurodegeneration Mechanisms
- Biochemical Acid Research Studies
- Prion Diseases and Protein Misfolding
- Alzheimer's disease research and treatments
- Cancer-related gene regulation
- Cholinesterase and Neurodegenerative Diseases
- Spinal Cord Injury Research
- Genetic Neurodegenerative Diseases
- Pharmacological Effects and Toxicity Studies
- Peripheral Neuropathies and Disorders
- Muscle Physiology and Disorders
- S100 Proteins and Annexins
- Acute Ischemic Stroke Management
- Hereditary Neurological Disorders
- Dysphagia Assessment and Management
- Autism Spectrum Disorder Research
- Erythropoietin and Anemia Treatment
- Autoimmune Neurological Disorders and Treatments
- Epigenetics and DNA Methylation
- Lysosomal Storage Disorders Research
Hanyang University
2015-2025
Hanyang University Medical Center
2016-2025
Hanyang University Seoul Hospital
2014-2024
Anyang University
2016-2023
Nagoya University
2020
National Institute of Public Health
2020
The University of Sydney
2020
Chiba University
2020
Royal Prince Alfred Hospital
2020
Geelong Hospital
2020
Abstract Stem cell therapy is an emerging alternative therapeutic or disease-modifying strategy for amyotrophic lateral sclerosis (ALS). The aim of this open-label phase I clinical trial was to evaluate the safety two repeated intrathecal injections autologous bone marrow (BM)-derived mesenchymal stromal cells (MSCs) in ALS patients. Eight patients with definite probable were enrolled. After a 3-month lead-in period, MSCs isolated times from BM at interval 26 days and then expanded vitro 28...
Objective To assess the safety and efficacy of 2 repeated intrathecal injections autologous bone marrow–derived mesenchymal stem cells (BM‐MSCs) in amyotrophic lateral sclerosis (ALS). Methods In a phase randomized controlled trial (NCT01363401), 64 participants with ALS were randomly assigned treatments (1:1) riluzole alone (control group, n = 31) or combined BM‐MSC (MSC 33). Safety was assessed based on occurrence adverse events. The primary outcome changes Amyotrophic Lateral Sclerosis...
Abstract The regulation of microglial cell phenotype is a potential therapeutic intervention in neurodegenerative disease. Previously, we reported that transforming growth factor-β (TGF-β) levels mesenchymal stromal cells (MSCs) could be used as biological markers to predict the effectiveness autologous MSC therapy patients with amyotrophic lateral sclerosis. However, underlying mechanism TGF-β MSCs was not fully elucidated determining functional properties microglia. In this study, aimed...
Objective This study aimed to determine the incidence, prevalence and survival time of Korean patients with amyotrophic lateral sclerosis (ALS) using National Health Insurance Service (NHIS) data. Methods Using NHIS data, nationwide health dataset, we identified motor neuron diseases who were first diagnosed a KCD-6 code (G12.20–G12.28; modified from ICD-10 codes) between 2011 2015. ALS (G12.21 code) epidemiological characteristics, including annual prevalence, mortality rates time, analysed...
Bone marrow mesenchymal stromal cells (MSCs) can modify disease progression in amyotrophic lateral sclerosis (ALS) model. However, there are currently no accurate biological markers for predicting the efficacy of autologous MSC transplants ALS patients. This open-label, single-arm, investigator-initiated clinical study was designed to identify MSCs that could be used as potential predictors response therapy patients with ALS. We enrolled 37 who received via intrathecal injection two monthly...
ANXA11 dysfunction induces alteration of intracellular Ca 2+ homeostasis and stress granule disassembly in ALS.
To establish whether amyotrophic lateral sclerosis (ALS) is a multistep process in South Korean and Japanese populations when compared to Australian cohorts.We generated incident data by age sex for (collected between April 2009 March 2010) patients with ALS January 2011 December 2015). Mortality rates were provided 2007 2016). We regressed the log of age-specific incidence against least squares regression each population.We identified 11,834 cases from 3 populations, including 6,524...
Abstract Amyotrophic lateral sclerosis (ALS) is characterized by selective and progressive neurodegenerative changes in motor neural networks. Given the system complexity, including anatomically distributed sites of degeneration from cortex to spinal cord chronic pro-inflammatory conditions, a cell-based therapeutic strategy could be an alternative approach treating ALS. Lessons previous mesenchymal stromal/stem cell (MSC) trials ALS realized importance 3 aspects current future MSC therapy,...
In a previous study, we reported that intrathecal injection of mesenchymal stem cells (MSCs) slowed disease progression in G93A mutant superoxide dismutase1 transgenic mice. this found MSC administration vastly increased the infiltration peripheral immune into spinal cord Amyotrophic lateral sclerosis (ALS) mice (G93A transgenic). Thus, investigated immunomodulatory effect MSCs on blood mononuclear (PBMCs) ALS patients, focusing regulatory T lymphocytes (Treg ; CD4(+) /CD25(high) /FoxP3(+) )...
The objective of this study was to compare the cost illness amyotrophic lateral sclerosis (ALS) in Korean population based on staging system for ALS from perspective both patients and government. Direct medical costs, care-related loss productivity with were measured records face-to-face interviews. divided into groups according ALS, analysed. A total 151 enrolled study. mean monthly US $7902 per patient increased disease stage (stage 2, $5181; 3, $7089; 4, $10,557). Of direct costs (US...
Objective The only identified cause of amyotrophic lateral sclerosis (ALS) are mutations in a number genes found familial cases but also sporadic cases. De novo occurring parental gonadal cell, the zygote or postzygotic during embryonal development can result an apparently sporadic/isolated case ALS later life. We searched for de SOD1 as ALS. Methods analysed peripheral-blood exome, genome and Sanger sequencing to identify deleterious 4000 patients from Germany, South Korea Sweden. Parental...
Background Reliable biomarkers are essential for predicting the progression speed and prognosis of patients with amyotrophic lateral sclerosis (ALS). We previously identified NCK-associated protein 1 (NCKAP1) as a critical factor in defective phagocytosis observed induced microglia-like cells (iMGs) from rapidly progressive sporadic ALS. This study explored roles microRNA (miRNA)-214, which targets NCKAP1 gene, Methods The discovery cohort (n=29) was used to identify miR-214 targeting genes....
Background Cognitive impairment is associated with a negative prognosis in amyotrophic lateral sclerosis (ALS), as well clinical specificity. We investigate neuropsychological function ALS patients without known genetic mutations Korean tertiary clinic. Methods Three hundred and eighteen were enrolled prospective longitudinal cohort from September 2008 to February 2012. At the time of diagnosis sporadic ALS, we carried out comprehensive tests on all patients, collected demographic...
Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. However, no reliable biomarkers have been identified to represent the clinical status. This study aimed investigate whether diffusion tensor imaging (DTI) findings are useful indicate status of ALS patients. Ninety-six probable or definite cases and 47 age- sex-matched, normal controls were enrolled. Demographic data collected at time DTI. DTI acquired using 3-Tesla magnetic resonance scanner analysed by voxel-wise...
It has been suggested that oxidative stress is one of the pathomechanisms underlying amyotrophic lateral sclerosis (ALS), and thus antioxidants such as uric acid (UA) could reduce might be beneficial in prevention or treatment this disease. The objective study was to prospectively investigate serum UA levels Korean ALS patients relate them disease progression. healthy controls who were individually well-matched for sex, age, body mass index (BMI) underwent blood testing levels, analyzed...
Objective Distal myopathy is a heterogeneous group of muscle diseases characterized by predominant distal weakness. A study was done to identify the underlying cause autosomal recessive adolescent onset myopathy. Methods Four patients from 2 unrelated Korean families were evaluated. To isolate genetic cause, exome sequencing performed. In vitro and in vivo assays using myoblast cells zebrafish models performed examine ADSSL1 mutation causing pathogenesis. Results Patients had an phenotype...
Mutations in the fused sarcoma (FUS) gene have been linked to amyotrophic lateral sclerosis (ALS). ALS patients with FUS mutations exhibit neuronal cytoplasmic mislocalization of mutant protein. patients’ fibroblasts or induced pluripotent stem cell (iPSC)-derived neurons developed as models for understanding ALS-associated (ALS-FUS) pathology; however, pathological signatures are not sufficiently present patients, whereas generation iPSC-derived from requires relatively intricate...
// Su Min Lim 1,2,* , Byung-Ok Choi 3,* Seong-il Oh 4,* Won Jun 5 Ki-Wook 2,6 Minyeop Nahm 2 Yuanchao Xue 7 Jae Hyeok Ji Young Young-Eun Kim 8 Ki Wha Chung 9 Xiang-Dong Fu 10 Chang-Seok 11 and Seung Hyun 1 Biomedical Research Institute, Hanyang University, Seoul, Republic of Korea Cell Therapy Center, University Hospital, 3 Department Neurology Neuroscience Samsung Medical Sungkyunkwan School Medicine, 4 Neurology, Busan Paik Inje College Busan, Sheikh Khalifa Specialty Ras Al Khaimah,...
Objective The objective of this study was to investigate the usefulness muscle ultrasound in evaluating dissociated small hand atrophy, termed ‘split hand’, and its feasibility diagnosis amyotrophic lateral sclerosis (ALS). Methods Forty-four patients with ALS, 18 normal subjects 9 other neuromuscular disorders were included study. muscles divided into three regions, median-innervated group (ML), ulnar-innervated (UL) medial (UM), echo intensity (EI) compound action potential (CMAP)...
With emerging evidence on the importance of non-cell autonomous toxicity in neurodegenerative diseases, therapeutic strategies targeting modulation key immune cells. including microglia and Treg cells, have been designed for treatment ALS other diseases. Strategy switching patient's environment from a pro-inflammatory toxic to an anti-inflammatory, neuroprotective condition, could be potential therapy Mesenchymal stem cells (MSCs) regulate innate adaptive through release soluble factors such...