A5032539741- Japanese History and Culture
- Chinese history and philosophy
- Neurological diseases and metabolism
- Indian and Buddhist Studies
- Prion Diseases and Protein Misfolding
- Genetic Neurodegenerative Diseases
- Alzheimer's disease research and treatments
- Parkinson's Disease Mechanisms and Treatments
- 14-3-3 protein interactions
- Amyotrophic Lateral Sclerosis Research
- Orthopedic Infections and Treatments
- Neurological disorders and treatments
- Infectious Diseases and Tuberculosis
- Peripheral Neuropathies and Disorders
- Mindfulness and Compassion Interventions
- Vietnamese History and Culture Studies
- Muscle Physiology and Disorders
- Cellular transport and secretion
- Peripheral Nerve Disorders
- Religion and Society Interactions
- Nuclear Receptors and Signaling
- Animal testing and alternatives
- Folate and B Vitamins Research
- Spinal Hematomas and Complications
- Twentieth Century Scientific Developments
University of Hawaiʻi at Mānoa
2012-2024
University of Hawaii System
2018
Hôpitaux Universitaires de Strasbourg
1996-2015
University of Hawaii–West Oahu
2014
Hôpital Civil, Strasbourg
2004-2010
Université de Strasbourg
2008-2010
Hôpital d'Hautepierre
2003-2010
Giessen School of Theology
2007
Kerckhoff Klinik
2007
Max Planck Institute for Biophysical Chemistry
2007
Gerstmann-Sträussler-Scheinker disease (GSS) is a cerebral amyloidosis associated with mutations in the prion protein (PrP) gene (PRNP). The aim of this study was to characterize amyloid peptides purified from brain tissue patient A117V mutation who Met/Val heterozygous at codon 129, Val129 being coupling phase mutant Val117. major peptide extracted fibrils ∼7-kDa PrP fragment. Sequence analysis and mass spectrometry showed that fragment had ragged N C termini, starting mainly Gly88 Gly90...
<h3>Objective</h3> To describe 2 unrelated patients with novel variations in the<i>POLG1</i>gene and features undistinguishable from multiple sclerosis, ie, optic neuritis, brain white matter hyperintense areas, unmatched cerebrospinal fluid oligoclonal bands. <h3>Design</h3> Case report. <h3>Setting</h3> University hospital. <h3>Patients</h3> Both subsequently developed bilateral ophthalmoplegia, ptosis, myopathy, cardiomyopathy, ataxia, dysphagia, hearing cognitive impairment. <h3>Main...
Abstract Lysosome‐associated membrane protein‐2 deficiency (LAMP‐2 deficiency), or Danon disease, is a rare X‐linked lysosomal disease characterized by cardiomyopathy, vacuolar myopathy, and mental retardation. Less than 20 families with mutations of the Lamp‐2 gene have been reported. We describe family from Sardinia eight affected patients (4 females 4 males) novel mutation in exon 2 (c.102_103delAG). Females developed isolated cardiomyopathy adulthood, whereas males presented retardation...
<i>Background:</i> The role of the 43-kDa transactivation-responsive DNA-binding protein (TDP43) in neurodegenerative diseases is not yet clearly established. <i>Objective:</i> To assess for first time presence TDP43 a patient with motor neuron disease (MND) and Parkinson’s (PD). <i>Methods:</i> A 78-year-old woman developed poorly dopa-responsive parkinsonism without cognitive alteration. Three years later, MND appeared led to death less than year....
Autocatalytic cleavage of lithostathine leads to the formation quadruple-helical fibrils (QHF-litho) that are present in Alzheimer's disease. Here we show such also occur Creutzfeldt-Jakob and Gerstmann-Sträussler-Scheinker diseases, where they form protease-K-resistant deposits co-localize with amyloid plaques formed from prion protein. Lithostathine does not appear change its native-like, globular structure during fibril formation. However, obtained evidence a cluster six conserved...
We report two patients with orthotopic liver transplantation (OLT) who developed a syndrome that fulfilled criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). One patient had OLT because of alcoholic cirrhosis and one following hepatitis C-induced hepatic failure. Both immunosuppressive therapy, cyclosporine prednisolone in case tacrolimus the other case. Treatment intravenous immune globulin (IVIG) significantly improved neuropathy both patients. In...
<i>Background/Aims:</i> Alzheimer’s disease (AD) is characterized by extracellular Aβ peptide deposition originating from amyloid precursor protein cleavage and intracellular neurofibrillary tangles resulting pathological tau aggregation. These processes are accompanied dramatic neuronal losses, further leading to different cognitive impairments. Neuronal death signalings involve gene expression modifications that rely on transcription factor alterations. Herein, we investigated...
Abstract We report three patients with a syndrome that fulfilled clinical and laboratory criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) who failed immunosuppressive treatment eventually developed progressive amyotrophic lateral sclerosis (ALS). Mean disease duration was 23 months (13–38) before death. Two had family history of ALS without mutations the SOD1 gene. Postmortem examination in one patient showed an endoneurial infiltration mononuclear cells...
The transformation of Buddhism during the Tokugawa period has not been suf≥ciently explored by modern scholars. In this essay I will attempt to sketch an overall view Tokugawa-period sectarian consciousness as expressed in relations between various obediences what is popularly called “the Zen sect,” namely Soto, Rinzai, and Obaku schools. question lineage identity central importance here, issue intimately connected with developments period, thus way which Japanese sects themselves today. By...