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The dysfunction of TAR DNA-binding protein-43 (TDP-43) is implicated in neurodegenerative diseases. However, the function of TDP-43 is not fully elucidated. Here we show that the protein level of endogenous TDP-43 in the nucleus is increased in mouse cortical neurons in the early stages, but return to basal level in the later stages after glutamate(More)
OBJECTIVE To explore the clinical features of a novel glycyl-tRNA synthetase (GARS) gene mutation in a family with Charcot-Marie-Tooth disease type 2D (CMT2D). METHODS Exome capture with the next-generation sequencing technique was used to detect gene mutations. The mutations were verified by the polymerase chain reaction-single strand conformation(More)
This study analyzed the clinical characteristics and outcomes of sacral extradural spinal meningeal cysts with spinal nerve root fibers treated by reconstruction of the nerve root sheaths. The relationships between the cysts and spinal nerve root fibers were examined microscopically, the cysts were partially excised, and the defects were oversewn to(More)
Ankyrin repeat, one of the most important protein motifs, plays a wide variety of roles in protein-protein interactions and in the signal pathways. Via large-scale sequencing, a novel 941-bp gene was isolated from an 18-week old human fetal brain cDNA library. It encodes a putative protein of 158 amino acid residues with four conserved ankyrin repeat(More)
Presence of susceptibility sign on middle cerebral artery (MCA) in T2 (∗) -weighted magnetic resonance (MR) images has been reported to detect acute MCA thromboembolic occlusion. However, the pathophysiologic course of thrombotic MCA occlusion differs from embolic occlusion, which might induce different imaging characters. Our study found that the(More)
This prospective study compares different clinical characteristics and outcomes of patients with two types of sacral extradural spinal meningeal cysts (SESMC) undergoing different means of surgical excision. Using the relationship between the cysts and spinal nerve roots fibers (SNRF) as seen under microscope, SESMCs were divided into two types: cysts with(More)
Abnormal TAR DNA-binding protein 43 (TDP-43) inclusion bodies can be detected in the degenerative neurons of amyotrophic lateral sclerosis. In this study, we induced chronic oxidative stress injury by applying malonate to cultured mouse cortical motor neurons. In the later stages of the malonate insult, TDP-43 expression reduced in the nuclei and(More)
OBJECTIVE To investigate the relationship between different distribution of NMDA receptor subunits and motor neurons selective vulnerability in amyotrophic lateral sclerosis. METHODS First we investigated whether administration of THA, a glutamate transport inhibitor, in cultured organotypic brain tissue made different damage of motor cortex and piriform(More)
OBJECTIVE To establish an in vitro model of amyotrophic lateral sclerosis (ALS) from the organotypic culture of SD rats' lumber spinal cord induced by the mitochondrial inhibitor,malonate sodium. METHOD The lumber spinal cord prepared from the 6-day-old SD rats was cut into 350 microm coronarily, cultured on the Millicell-CM inserts which make the spinal(More)