A5031994898- Lysosomal Storage Disorders Research
- Virus-based gene therapy research
- Cytomegalovirus and herpesvirus research
- Viral Infectious Diseases and Gene Expression in Insects
- Cellular transport and secretion
- Pancreatic and Hepatic Oncology Research
- Neuroblastoma Research and Treatments
- Traumatic Brain Injury Research
- CRISPR and Genetic Engineering
- Genetics, Aging, and Longevity in Model Organisms
- Phosphodiesterase function and regulation
- FOXO transcription factor regulation
- Mineral Processing and Grinding
- Bone Tissue Engineering Materials
- Integrated Circuits and Semiconductor Failure Analysis
- Protein purification and stability
- Acute Ischemic Stroke Management
- Granular flow and fluidized beds
- Calcium signaling and nucleotide metabolism
- Phagocytosis and Immune Regulation
- Mitochondrial Function and Pathology
- Biomedical Text Mining and Ontologies
- Ethics in medical practice
- Semiconductor materials and devices
- Hereditary Neurological Disorders
Taipei Medical University-Shuang Ho Hospital
2023-2025
The University of Texas Health Science Center at Houston
2023
University of Houston
2023
Wake Forest University
2023
Cornell University
2013-2023
Weill Cornell Medicine
2023
King's College Hospital
2022
National Tsing Hua University
2022
Philips (United States)
2021
Chang Gung Memorial Hospital
2021
Alzheimer's disease (AD) is a progressive degenerative neurological disorder affecting nearly one in nine elderly people the United States. Population studies have shown that an inheritance of apolipoprotein E (APOE) variant APOE4 allele increases risk developing AD, whereas APOE2 homozygotes are protected from late-onset AD. It was hypothesized expression "protective" by genetic modification central nervous system (CNS) could reverse or prevent neurologic damage. To assess CNS distribution...
Lactate dehydrogenase A (LDHA) can regulate tumorigenesis and cancer progression. Nevertheless, whether the regulation of LDHA is involved in development gemcitabine resistance PDAC has not yet been fully elucidated. Increasing studies have shown that acquired drug led to treatment failure highly attributed stem cell (CSC) properties. Therefore, we aim demonstrate functions regulatory mechanisms on properties PDAC. We investigate metabolite profiles by liquid chromatography-mass spectrometry...
Abstract Changes in mitochondrial dynamics (fusion and fission) are known to occur during stem cell differentiation; however, the role of this phenomenon tissue aging remains unclear. Here, we report that shifted toward fission Drosophila ovarian germline cells (GSCs), shift contributes aging‐related GSC loss. We found as GSCs age, fragmentation expression regulator, Dynamin‐related protein (Drp1), both increased, while membrane potential is reduced. Moreover, preventing fusion results...
Administration of an adeno-associated virus coding for the CLN2 gene into brain parenchyma slows progression disease in children.
Metachromatic leukodystrophy (MLD), a fatal disorder caused by deficiency of the lysosomal enzyme arylsulfatase A (ARSA), is associated with an accumulation sulfatides, causing widespread demyelination in both central and peripheral nervous systems. On basis prior studies demonstrating that adeno-associated virus AAVrh.10 can mediate distribution CNS secreted transgene, as prelude to human trials, we comparatively assessed optimal delivery route vector encoding ARSA large animal model for...
Introduction: Neuronal ceroid lipofuscinoses (NCL) are neurodegenerative lysosomal storage disorders typically characterized by cognitive and visual impairments, epileptic seizures, ataxia, deterioration of motor skills. Recent success Brineura® for the treatment neurologic manifestations CLN2 disease has led to renewed interest in therapeutics NCL. Despite complex challenges associated with CNS therapy, modalities such as enzyme replacement gene stem cell small molecule pharmacotherapy have...
Juvenile neuronal ceroid lipofuscinosis (JNCL or CLN3 disease) is an autosomal recessive lysosomal storage disease resulting from mutations in the gene that encodes a membrane protein. The primarily affects brain with widespread intralysosomal accumulation of autofluorescent material and fibrillary gliosis, as well loss specific populations. As experimental treatment for CNS manifestations JNCL, we have developed serotype rh.10 adeno-associated virus vector expressing human cDNA...
A method is presented for quantitative analysis of the biodistribution adeno-associated virus (AAV) gene transfer vectors following in vivo administration. We used iodine-124 (I-124) radiolabeling AAV capsid and positron emission tomography combined with compartmental modeling to quantify whole-body organ-specific capsids from 1 72 h Using intravenous (IV) intracisternal (IC) routes administration AAVrh.10 AAV9 nonhuman primates absence or presence anticapsid immunity, we have identified...
Metachromatic leukodystrophy, a fatal pediatric neurodegenerative lysosomal storage disease caused by mutations in the arylsulfatase A (ARSA) gene, is characterized intracellular accumulation of sulfatides lysosomes cells central nervous system (CNS). In previous studies, we have demonstrated efficacy AAVrh.10hARSA, an adeno-associated virus (AAV) serotype rh.10 vector coding for human ARSA gene to CNS mouse model disease, and that catheter-based intraparenchymal administration AAVrh.10hARSA...
Colorectal cancer is the second most common cause of death from in UK. It estimated that between 2 to 3 per cent colorectal occurs patients younger than age 40 years. remains unclear literature whether this group has a worse prognosis population as whole. There are no large series report 10-year survival young diagnosed with cancer. The authors’ objective was assess years determine 5- and rates tertiary referral center compares favorably obtained at other centers A retrospective...
In this paper, an industry-level new-generation EDA solution for reliability-aware design in nanoscale FinFET technology is presented the first time, with new compact transistor aging models and upgraded circuit reliability simulator. Our work solves various issues found silicon data of NBTI aging. Especially, instead ignoring or less accurate recovery effect model traditional simulators, degradation are proposed validated by full stress/recovery range technology. The history effect, one...
Friedreich's ataxia (FA) is a life-threatening autosomal recessive disorder characterized by neurological and cardiac dysfunction. Arrhythmias heart failure are the main cause of premature death. From prior studies in murine models FA, adeno-associated virus encoding normal human frataxin gene (AAVrh.10hFXN) effectively treated manifestations disease. However, therapeutic dose window limited high level (hFXN) expression associated with toxicity. As goal, since FA heterozygotes have no...
Aging | doi:10.18632/aging.204867. Tung-Wei Hsu, Yen-Hao Su, Hsin-An Chen, Po-Hsiang Liao, Shih Chiang Shen, Kuei-Yen Tsai, Tzu-Hsuan Wang, Alvin Chih-Yang Huang, Marthandam Asokan Shibu, Wan-Yu Shing-Chuan Shen
α1-antitrypsin (AAT) deficiency is a common autosomal recessive hereditary disorder, with high risk for the development of early-onset panacinar emphysema. AAT, produced primarily in liver, functions to protect lung from neutrophil protease; AAT deficiency, unimpeded proteases destroy parenchyma. susceptible oxidative damage resulting an inability inhibit its target proteases, elastase, and cathepsin G. The major sites modification on molecule are methionine residues 351 358. We have...
<h3>Purpose:</h3> To determine whether an immediate referral to a medical-legal partnership (MLP), compared with 6-month waitlist control, improved mental health, health care use, and quality of life. <h3>Methods:</h3> This trial randomly assigned individuals or wait-list control. The MLP involved collaboration between the primary clinic legal services organization. outcome was stress (6 months) as measured by Perceived Stress Scale (PSS). Secondary measures included Center for Epidemiologic...
Growing demand for energy-efficient, high-performance systems has resulted in the growth of innovative heterogeneous computing system architectures that use FPGAs. FPGA-based enable designers to implement custom instruction streams executing on potentially thousands compute elements. Traditionally, FPGAs have been used as elements PCI devices; however, this does not allow be co-processors. This paper describes a architecture is based Intel® Xeon® platform which one or more FPGAs, acting...
The development of a drug product requires rigorous methods characterization and quality control to assure potency. Gene therapy products, relatively new strategy for design with very few licensed examples, represent unique challenge the measure Unlike traditional drugs, potency gene therapeutic is tally measures multiple steps, including infectivity, transcription, translation, protein modifications, proper localization product, function. This particularly challenging products based on...
Abstract High‐grade neuroendocrine tumors (NETs) of the lung consist small‐cell cancer (SCLC) and large‐cell carcinoma (LCNEC). Both exhibit aggressive malignancy with poor prognosis. The transformation adenocarcinoma (ADC) to SCLC or LCNEC also contributes acquired resistance epidermal growth factor receptor (EGFR)‐tyrosine kinase inhibitors (TKIs). Despite initially being responsive chemotherapy, high‐grade NET patients inevitably develop drug resistance; thus, novel therapeutic targets...
Chronic traumatic encephalopathy (CTE) is a progressive neurodegenerative disorder caused by repetitive trauma to the central nervous system (CNS) suffered soldiers, contact sport athletes, and civilians following accident-related trauma. CTE CNS tauopathy, with trauma-induced inflammation leading accumulation of hyperphosphorylated forms microtubule-binding protein Tau (pTau), resulting in neurofibrillary tangles loss neurons. At present, there are no therapies treat CTE. We hypothesized...
Although the effectiveness of robotic hepatectomy (RH) has been evaluated in several studies, superiority RH over other approaches not definitely established. Therefore, present propensity score-matched cohort study, we compared and laparoscopic (LH) terms perioperative oncologic outcomes.This retrospective study included patients who underwent or LH for benign malignant liver lesions at a single center Taiwan any time between 2014 2020. Confounding factors, specifically age, sex, body mass...
Metachromatic leukodystrophy (MLD), a lysosomal storage disorder caused by the deficiency of enzyme arylsulfatase A (ARSA), is recessive, fatal neurodegenerative disease associated with intracellular accumulation sulfatides in CNS. The fatal, no approved therapy. focus this study to test hypothesis that direct CNS administration AAVrh.10hARSA (AAVrh.10 serotype vector coding for human ARSA cDNA) nonhuman primates at doses scalable humans has an acceptable long term safety profile. Safety...
Pancreatic cancer has one of the highest fatality rates and poorest prognosis among all types worldwide. Gemcitabine is a commonly used first-line therapeutic drug for pancreatic cancer; however, rapid development resistance to gemcitabine treatment been observed in numerous patients with cancer, this phenomenon limits survival benefit gemcitabine. Adenylosuccinate lyase (ADSL) crucial enzyme that serves dual functions de novo purine biosynthesis, it demonstrated be associated clinical...