Recently, C9orf72 hexanucleotide (GGGGCC) repeat expansion in intron 1 was reported to be the most common cause of sporadic and familial amyotrophic lateral sclerosis (ALS)/frontotemporal dementia (FTD) in the Caucasian population. The frequency of the intronic repeat expansion is up to 21%-57% in familial ALS and 3%-21% in sporadic ALS.In the Japanese population, the C9orf72 repeat expansion w...

Amyotrophic lateral sclerosis (ALS) is recognized as a motor neuron disorder affecting the structure and function of the brain and neuromuscular system. Very little is known, however, that the nervous system degeneration is dependent on disease phenotypes of ALS. The purpose of this study was to determine the degree of brain white matter (WM) structure degeneration in four ALS patient groups ch...

INTRODUCTION A decreased cerebrospinal fluid (CSF) p-Tau181 to total tau ratio (p/t-tau) is a biomarker for frontotemporal lobar degeneration with TDP43 inclusions (FTLD-TDP) and for amyotrophic lateral sclerosis (ALS). CSF light chain neurofilaments (NfL) are increased in ALS. We examined whether CSF p/t-tau and NfL are related to ALS status in FTLD-TDP. METHODS We compared CSF p/t-tau and N...

OBJECTIVES Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease, and sepsis is a frequent cause of death in hospitalised patients. We investigated the relationship between ALS and the subsequent risk of sepsis. DESIGN A retrospective cohort analysis. SETTING Patients with ALSs diagnosed between 2000 and 2010 in Taiwan National Health Insurance Research Database. PARTIC...

Although the copper/zinc superoxide dismutase-1 (SOD1) gene has been identified in both familial ALS (FALS) and sporadic ALS (SALS), it has rarely been studied in Chinese patients with ALS, and there are few studies with large samples. This study sought to assess the prevalence of SOD1 mutations in Chinese ALS patients. We screened a cohort of 499 ALS patients (487 SALS and 12 FALS) from the De...

Ovarian cancer is a leading killer of women, and no cure for advanced ovarian cancer is available. Alisertib (ALS), a selective Aurora kinase A (AURKA) inhibitor, has shown potent anticancer effects, and is under clinical investigation for the treatment of advanced solid tumor and hematologic malignancies. However, the role of ALS in the treatment of ovarian cancer remains unclear. This study i...

the dimerization constants of safranine t have been determined by studying the dependence of its absorption spectra on the temperature in the range 30–70 ◦c at different total concentrations of safranine t (1.03×10−5, 1.44×10−5 and 1.73×10−5 m). the monomer–dimer equilibrium of safranine t has been determined by applying mcr-als method on the absorption spectra. the quantitative analysis of the...

Amyotrophic lateral sclerosis (ALS) is a late-onset fatal neurodegenerative disease affecting motor neurons with an incidence of about 1/100,000. Most ALS cases are sporadic, but 5-10% of the cases are familial ALS. Both sporadic and familial ALS (FALS) are associated with degeneration of cortical and spinal motor neurons. The etiology of ALS remains unknown. However, mutations of superoxide di...

Little is known about global gene expression patterns in the human neurodegenerative disease amyotrophic lateral sclerosis (ALS). To address this, we used high-density oligonucleotide microarray technology to compare expression levels of approximately 6,800 genes in postmortem spinal cord gray matter obtained from individuals with ALS as well as normal individuals. Using Fisher discriminant ana...

Self-referential processing is a key cognitive process, associated with the serotonergic system and the default mode network (DMN). Decreased levels of serotonin and reduced activations of the DMN observed in amyotrophic lateral sclerosis (ALS) suggest that self-referential processing might be altered in patients with ALS. Here, we investigate the effects of ALS on the electroencephalography co...