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Chapter and Conference Paper
Live facial expression generation based on mixed reality
Virtual reality technology provides a new methodology for visualization with realistic sensation, and has attracted special interests of human interface, visual communication communities. The key issue there i...
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Article
High-quality crystal growth and characterization of organic nonlinear optical crystal: 4-dimethylamino-N-methyl-4-stilbazolium tosylate (DAST)
High quality 4-dimethylamino-N-methyl-4-stilbazolium tosylate (DAST) crystal has been successfully grown from saturated methanol solution by a slow cooling method in a Teflon vessel. The full width at half max...
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Article
Leuprorelin rescues polyglutamine-dependent phenotypes in a transgenic mouse model of spinal and bulbar muscular atrophy
Spinal and bulbar muscular atrophy (SBMA) is an adult-onset motor neuron disease that affects males. It is caused by the expansion of a polyglutamine (polyQ) tract in androgen receptors. Female carriers are us...
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Article
Sweet relief for Huntington disease
Oral delivery of a simple, nontoxic sugar molecule alleviates symptoms of Huntington disease in a mouse model (pages 148–154).
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Article
Spinal and bulbar muscular atrophy: ligand-dependent pathogenesis and therapeutic perspectives
Spinal and bulbar muscular atrophy (SBMA) is a late-onset motor neuron disease characterized by proximal muscle atrophy, weakness, contraction fasciculations, and bulbar involvement. SBMA exclusively affects m...
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Article
17-AAG, an Hsp90 inhibitor, ameliorates polyglutamine-mediated motor neuron degeneration
Heat-shock protein 90 (Hsp90) functions as part of a multichaperone complex that folds, activates and assembles its client proteins. Androgen receptor (AR), a pathogenic gene product in spinal and bulbar muscu...
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Article
Classic Polyarteritis Nodosa Presenting Rare Clinical Manifestations in a Patient with Hemophilia A
A 35-year-old patient with hemophilia A presented with rapidly progressive polyarteritis nodosa (PAN). He had been infected with hepatitis B virus (HBV) by repeated transfusion and was positive for hepatitis B...
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Article
Modulation of Hsp90 function in neurodegenerative disorders: a molecular-targeted therapy against disease-causing protein
Abnormal accumulation of disease-causing protein is a commonly observed characteristic in chronic neurodegenerative disorders such as Alzheimer’s disease, Parkinson’s disease, and polyglutamine (polyQ) disease...
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Article
Laser energy dependence on femtosecond laser-induced nucleation of protein
We have investigated femtosecond laser-induced nucleation of hen egg-white lysozyme (HEWL) as a function of the laser pulse energy and pulse time width. This is the first recorded study to confirm that the fem...
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Article
Getting a handle on Huntington's disease: the case for cholesterol
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Chapter
Clinical Features and Molecular Mechanisms of Spinal and Bulbar Muscular Atrophy (SBMA)
Spinal and bulbar muscular atrophy (SBMA) is an adult-onset neurodegenerative disease characterized by slowly progressive muscle weakness and atrophy. The cause of this disease is the expansion of ...
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Article
Complement-mediated bacteriolysis after binding of specific antibodies to drug-resistant Pseudomonas aeruginosa: morphological changes observed by using a field emission scanning electron microscope
A bactericidal mechanism mediated by human serum was investigated by a field emission scanning electron microscope and a strain of drug-resistant Pseudomonas aeruginosa. When the bacteria were treated with merope...
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Article
Viral delivery of miR-196a ameliorates the SBMA phenotype via the silencing of CELF2
Spinal and bulbar muscular atrophy (SBMA) is a neurodegenerative disease caused by aggregation of the androgen receptor (AR) protein. Here Gen Sobue and colleagues show that upregulation of miR-196a can reduce...
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Article
Naratriptan mitigates CGRP1-associated motor neuron degeneration caused by an expanded polyglutamine repeat tract
Spinal and bulbar musclar atrophy (SBMA) is caused by expanded polyglutamine repeats in the androgen receptor, leading to motor neuron degeneration. Gen Sobue and his colleagues describe a molecular cascade wh...
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Article
Heat shock factor-1 influences pathological lesion distribution of polyglutamine-induced neurodegeneration
A crucial feature of adult-onset neurodegenerative diseases is accumulation of abnormal protein in specific brain regions, although the mechanism underlying this pathological selectivity remains unclear. Heat ...
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Protocol
In Vivo Phosphorylation of WRKY Transcription Factor by MAPK
Plants activate signaling networks in response to diverse pathogen-derived signals, facilitating transcriptional reprogramming through mitogen-activated protein kinase (MAPK) cascades. Identification of phosph...
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Article
Open AccessFUS/TLS acts as an aggregation-dependent modifier of polyglutamine disease model mice
FUS/TLS is an RNA/DNA-binding protein associated with neurodegenerative diseases including amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Previously, we found that a prion-like domain in ...
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Article
Promotion of protein crystal growth by actively switching crystal growth mode via femtosecond laser ablation
The protein crystal growth mechanism can be changed from planar 2D nucleation growth to spiral growth by femtosecond laser ablation. By using this method, the growth rate of a hen egg-white lysozyme crystal in...
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Article
Zebra sign of precentral gyri in amyotrophic lateral sclerosis: A novel finding using phase difference enhanced (PADRE) imaging-initial results
We compared the precentral gyri (PG) on the PADRE of patients with amyotrophic lateral sclerosis (ALS) and healthy subjects (HSs) in order to determine whether it is possible to discriminate between ALS patien...
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Article
Open AccessSystemic overexpression of SQSTM1/p62 accelerates disease onset in a SOD1H46R-expressing ALS mouse model
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by a selective loss of upper and lower motor neurons. Recent studies have shown that mutations in SQSTM1 are linked to ...
