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Amyotrophic lateral sclerosis (ALS) has its onset in middle age and is a progressive disorder characterized by degeneration of motor neurons of the primary motor cortex, brainstem and spinal cord. Most cases of ALS are sporadic, but about 10% are familial. Genes known to cause classic familial ALS (FALS) are superoxide dismutase 1 (SOD1), ANG encoding(More)
Mutations in the fused in sarcoma (FUS, also known as translated in liposarcoma) gene have been recently discovered to be associated with familial amyotrophic lateral sclerosis (FALS) in African, European and American populations. In a Japanese family with FALS, we found the R521C FUS mutation, which has been reported to be found in various ethnic(More)
Forkhead box O (Foxo) transcription factors induce muscle atrophy by upregulating the muscle-specific E3 ubiquitin ligases MuRF-1 and atrogin-1/MAFbx, but other than Akt, the upstream regulators of Foxos during muscle atrophy are largely unknown. To examine the involvement of the dystrophin glycoprotein complex (DGC) in regulation of Foxo activities and(More)
Sporadic inclusion body myositis (sIBM) is the most common form of myopathy with inflammation in those over the age of 50 years in Western countries [1, 3, 5, 7]. The prevalence in Caucasians is 4.9–14.9 per million, but 1.07 in Turkey [6]. The prevalence of sIBM in Asian people including Japanese has not been examined. Several mechanisms of sIBM are(More)
OBJECTIVE The feasibility of human cardiac imaging using a prototype 256-detector row cone-beam computed tomography (256CBCT) scanner without electrocardiographic gating was examined. METHODS Two healthy male volunteers were examined by contrast-enhanced 256CBCT. The number of detectors was 912 x 256, each measuring approximately 0.5 mm x 0.5 mm at the(More)
Using transgenic mice harboring a targeted LacZ insertion, we studied the expression pattern of the C9ORF72 mouse ortholog (3110043O21Rik). Unlike most genes that are mutated in amyotrophic lateral sclerosis (ALS), which are ubiquitously expressed, the C9ORF72 ortholog was most highly transcribed in the neuronal populations that are sensitive to(More)
Basophilic inclusions (BIs) are pathological features of a subset of frontotemporal lobar degeneration disorders, including sporadic amyotrophic lateral sclerosis (ALS) and familial ALS (FALS). Mutations in the fused in sarcoma/translocated in liposarcoma (FUS/TLS) gene have recently been identified as a cause of FALS. The FUS/TLS-immunoreactive inclusions(More)
PURPOSE To evaluate the clinical outcomes of cantilever transforaminal lumbar interbody fusion (c-TLIF) for upper lumbar diseases. MATERIALS AND METHODS Seventeen patients (11 males, 6 females; mean ± SD age: 62 ± 14 years) who underwent c-TLIF using kidney type spacers between 2002 and 2008 were retrospectively evaluated, at a mean follow-up of 44.1 ±(More)
Myofibrillar myopathy (MFM) is a group of chronic muscular disorders that show the focal dissolution of myofibrils and accumulation of degradation products. The major genetic basis of MFMs is unknown. In 1993, our group reported a Japanese family with dominantly inherited cytoplasmic body myopathy, which is now included in MFM, characterized by late-onset(More)
Although deep hip bending activities are often required in Asian populations because of traditional lifestyles and religious practices, few have examined the required hip range of motion (ROM) in these activities after total hip arthroplasty (THA). We performed postoperative motion analysis to evaluate the differences in required ROMs between Japanese-style(More)