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Phosphorylated TDP‐43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
The aim of this study was to identify the phosphorylation sites and responsible kinases, and to clarify the pathological significance ofosphorylation of TDP‐43.
Association of HTRA1 mutations and familial ischemic cerebral small-vessel disease.
The findings indicate a link between repressed inhibition of signaling by the TGF-beta family and ischemic cerebral small-vessel disease, alopecia, and spondylosis.
Neuroprotective Effects of Glial Cell Line-Derived Neurotrophic Factor Mediated by an Adeno-Associated Virus Vector in a Transgenic Animal Model of Amyotrophic Lateral Sclerosis
It is shown here that AAV-GDNF leads to substantial and long-lasting expression of transgenic GDNF in a large number of myofibers with its accumulation at the sites of neuromuscular junctions, indicating that A AV-mediated GDNF delivery to the muscle is a promising means of gene therapy for ALS.
A revision of the El Escorial criteria - 2015
There has been much discussion as to the necessity for adjustment of the El Escorial diagnostic criteria, primarily based on observations relating to the specificity of the‘Possible’ category. The ...
Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial
Location of the corticospinal tract in the internal capsule at MR imaging.
Normal hyperintense foci in the IC represent the fibers of the corticospinal tract and the presence of large fibers with thick myelin sheaths may play an important role in these signal intensity variations.
New consensus research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 (SOD1) gene mutations: Inclusions containing SOD1 in neurons and astrocytes
Based on the immuno-electron microscopical finding that these abnormal granulecoated fibrils are positive for SOD1, the formation (or aggregation) of the abnormal fibril containing S OD1 would be essential evidence in diseases caused by various SOD 1 mutations.
A Phase I Study of Aromatic L-Amino Acid Decarboxylase Gene Therapy for Parkinson's Disease.
- S. Muramatsu, K. Fujimoto, I. Nakano
- BiologyMolecular therapy : the journal of the American…
- 1 September 2010
The findings provide class IV evidence regarding the safety and efficacy of AADC gene therapy and warrant further evaluation in a randomized, controlled, phase 2 setting.
Heading Disorientation: A New Test and a Possible Underlying Mechanism
The results of the CPT suggest that HD patients cannot integrate information on the spatial locations of objects derived from an egocentric reference frame with that on changes of the body directions, and the retrosplenial cortex may be the place where these different types of information necessary for navigation converge.
Delayed delivery of AAV-GDNF prevents nigral neurodegeneration and promotes functional recovery in a rat model of Parkinson's disease
Significant behavioral recovery was observed from 4–20 weeks following AAV-GDNFflag injection, indicating that a delayed delivery of GDNF gene using AAV vector is efficacious even 4 weeks after the onset of progressive degeneration in a rat model of PD.