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Amyotrophic lateral sclerosis (ALS) is a rapidly progressive, devastating and fatal disease characterized by selective loss of upper and lower motor neurons of the cerebral cortex, brainstem, spinal cord and muscle atrophy. In spite of many years of research, the pathogenesis of ALS is still not well understood. ALS is a multifaceted genetic disease, in which genetic susceptibility to motor neuron death interacts with environmental factors and there is still no cure for this deleterious disease. At present, there is only one FDA approved drug, Riluzole which according to past studies only modestly slows the progression of the disease, and improves survival by up to three months. The suffering of the ALS patients, and their families is enormous and the economic burden is colossal. There is therefore a pressing need for new therapies. Different molecular pathways and pathological mechanisms have been implicated in ALS. According to past studies, altered calcium homeostasis, abnormal mitochondrial function, protein misfolding, axonal transport defects, excessive production of extracellular superoxide radicals, glutamate-mediated excitotoxicity, inflammatory events, and activation of oxidative stress pathways within the mitochondria and endoplasmic reticulum can act as major contributor that eventually leads to loss of connection between muscle and nerve ultimately resulting to ALS. However, the detailed molecular and cellular pathophysiological mechanisms and origin and temporal progression of the disease still remained elusive. Ongoing research and future advances will likely advance our improve understanding about various involved pathological mechanism ultimately leading to discoveries of new therapeutic cures. Importantly, clinical biomarkers of disease onset and progression are thus also urgently needed to support the development of the new therapeutic agents and novel preventive and curative strategies. Effective translation from pre-clinical to clinical studies will further require extensive knowledge regarding drug activity, bioavailability and efficacy in both the pre-clinical and clinical setting, and proof of biological activity in the target tissue. During the last decades, the development of new therapeutic molecules, advance neuroimaging tools, patient derived induced stem cells and new precision medicine approaches to study ALS has significantly improved our understanding of disease. In particular, new genetic tools, neuroimaging methods, cellular probes, biomarker study and molecular techniques that achieve high spatiotemporal resolution have revealed new details about the disease onset and its progression. In our effort to provide the interested reader, clinician and researchers a comprehensive summaries and new findings in this field of ALS research, hereby we have created this electronic book which comprises of twenty seven chapters having various reviews, perspective and original research articles. All these chapters and articles in this book not only summarize the cutting-edge techniques, approaches, cell and animal models to study ALS but also provide unprecedented coverage of the current developments and new hypothesis emerging in ALS research. Some examples are novel genetic and cell culture based models, mitochondria-mediated therapy, oxidative stress and ROS mechanism, development of stem cells and mechanism-based therapies as well as novel biomarkers for designing and testing effective therapeutic strategies that can benefit ALS patients who are at the earlier stages in the disease. I am extremely grateful to all the contributors to this book and want to thank them for their phenomenal efforts. Amyotrophic lateral sclerosis (ALS) is a rapidly progressive, devastating and fatal disease characterized by selective loss of upper and lower motor neurons of the cerebral cortex, brainstem, spinal cord and muscle atrophy. In spite of many years of research, the pathogenesis of ALS is still not well understood. ALS is a multifaceted genetic disease, in which genetic susceptibility to motor neuron death interacts with environmental factors and there is still no cure for this deleterious disease. At present, there is only one FDA approved drug, Riluzole which according to past studies only modestly slows the progression of the disease, and improves survival by up to three months. The suffering of the ALS patients, and their families is enormous and the economic burden is colossal. There is therefore a pressing need for new therapies. Different molecular pathways and pathological mechanisms have been implicated in ALS. According to past studies, altered calcium homeostasis, abnormal mitochondrial function, protein misfolding, axonal transport defects, excessive production of extracellular superoxide radicals, glutamate-mediated excitotoxicity, inflammatory events, and activation of oxidative stress pathways within the mitochondria and endoplasmic reticulum can act as major contributor that eventually leads to loss of connection between muscle and nerve ultimately resulting to ALS. However, the detailed molecular and cellular pathophysiological mechanisms and origin and temporal progression of the disease still remained elusive. Ongoing research and future advances will likely advance our improve understanding about various involved pathological mechanism ultimately leading to discoveries of new therapeutic cures. Importantly, clinical biomarkers of disease onset and progression are thus also urgently needed to support the development of the new therapeutic agents and novel preventive and curative strategies. Effective translation from pre-clinical to clinical studies will further require extensive knowledge regarding drug activity, bioavailability and efficacy in both the pre-clinical and clinical setting, and proof of biological activity in the target tissue. During the last decades, the development of new therapeutic molecules, advance neuroimaging tools, patient derived induced stem cells and new precision medicine approaches to study ALS has significantly improved our understanding of disease. In particular, new genetic tools, neuroimaging methods, cellular probes, biomarker study and molecular techniques that achieve high spatiotemporal resolution have revealed new details about the disease onset and its progression. In our effort to provide the interested reader, clinician and researchers a comprehensive summaries and new findings in this field of ALS research, hereby we have created this electronic book which comprises of twenty seven chapters having various reviews, perspective and original research articles. All these chapters and articles in this book not only summarize the cutting-edge techniques, approaches, cell and animal models to study ALS but also provide unprecedented coverage of the current developments and new hypothesis emerging in ALS research. Some examples are novel genetic and cell culture based models, mitochondria-mediated therapy, oxidative stress and ROS mechanism, development of stem cells and mechanism-based therapies as well as novel biomarkers for designing and testing effective therapeutic strategies that can benefit ALS patients who are at the earlier stages in the disease. I am extremely grateful to all the contributors to this book and want to thank them for their phenomenal efforts.

Mitochondria --- neurodegenerative disease --- Ca2+ signaling --- multidrug therapy --- Amyotrophic lateral sclerosis (ALS) --- Superoxide dismutase 1 (SOD1) --- Motor neuron disease (MND) --- excitotoxicity --- Riluzole --- multifactorial disease

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Amyotrophic lateral sclerosis (ALS) is a rapidly progressive, devastating and fatal disease characterized by selective loss of upper and lower motor neurons of the cerebral cortex, brainstem, spinal cord and muscle atrophy. In spite of many years of research, the pathogenesis of ALS is still not well understood. ALS is a multifaceted genetic disease, in which genetic susceptibility to motor neuron death interacts with environmental factors and there is still no cure for this deleterious disease. At present, there is only one FDA approved drug, Riluzole which according to past studies only modestly slows the progression of the disease, and improves survival by up to three months. The suffering of the ALS patients, and their families is enormous and the economic burden is colossal. There is therefore a pressing need for new therapies. Different molecular pathways and pathological mechanisms have been implicated in ALS. According to past studies, altered calcium homeostasis, abnormal mitochondrial function, protein misfolding, axonal transport defects, excessive production of extracellular superoxide radicals, glutamate-mediated excitotoxicity, inflammatory events, and activation of oxidative stress pathways within the mitochondria and endoplasmic reticulum can act as major contributor that eventually leads to loss of connection between muscle and nerve ultimately resulting to ALS. However, the detailed molecular and cellular pathophysiological mechanisms and origin and temporal progression of the disease still remained elusive. Ongoing research and future advances will likely advance our improve understanding about various involved pathological mechanism ultimately leading to discoveries of new therapeutic cures. Importantly, clinical biomarkers of disease onset and progression are thus also urgently needed to support the development of the new therapeutic agents and novel preventive and curative strategies. Effective translation from pre-clinical to clinical studies will further require extensive knowledge regarding drug activity, bioavailability and efficacy in both the pre-clinical and clinical setting, and proof of biological activity in the target tissue. During the last decades, the development of new therapeutic molecules, advance neuroimaging tools, patient derived induced stem cells and new precision medicine approaches to study ALS has significantly improved our understanding of disease. In particular, new genetic tools, neuroimaging methods, cellular probes, biomarker study and molecular techniques that achieve high spatiotemporal resolution have revealed new details about the disease onset and its progression. In our effort to provide the interested reader, clinician and researchers a comprehensive summaries and new findings in this field of ALS research, hereby we have created this electronic book which comprises of twenty seven chapters having various reviews, perspective and original research articles. All these chapters and articles in this book not only summarize the cutting-edge techniques, approaches, cell and animal models to study ALS but also provide unprecedented coverage of the current developments and new hypothesis emerging in ALS research. Some examples are novel genetic and cell culture based models, mitochondria-mediated therapy, oxidative stress and ROS mechanism, development of stem cells and mechanism-based therapies as well as novel biomarkers for designing and testing effective therapeutic strategies that can benefit ALS patients who are at the earlier stages in the disease. I am extremely grateful to all the contributors to this book and want to thank them for their phenomenal efforts. Amyotrophic lateral sclerosis (ALS) is a rapidly progressive, devastating and fatal disease characterized by selective loss of upper and lower motor neurons of the cerebral cortex, brainstem, spinal cord and muscle atrophy. In spite of many years of research, the pathogenesis of ALS is still not well understood. ALS is a multifaceted genetic disease, in which genetic susceptibility to motor neuron death interacts with environmental factors and there is still no cure for this deleterious disease. At present, there is only one FDA approved drug, Riluzole which according to past studies only modestly slows the progression of the disease, and improves survival by up to three months. The suffering of the ALS patients, and their families is enormous and the economic burden is colossal. There is therefore a pressing need for new therapies. Different molecular pathways and pathological mechanisms have been implicated in ALS. According to past studies, altered calcium homeostasis, abnormal mitochondrial function, protein misfolding, axonal transport defects, excessive production of extracellular superoxide radicals, glutamate-mediated excitotoxicity, inflammatory events, and activation of oxidative stress pathways within the mitochondria and endoplasmic reticulum can act as major contributor that eventually leads to loss of connection between muscle and nerve ultimately resulting to ALS. However, the detailed molecular and cellular pathophysiological mechanisms and origin and temporal progression of the disease still remained elusive. Ongoing research and future advances will likely advance our improve understanding about various involved pathological mechanism ultimately leading to discoveries of new therapeutic cures. Importantly, clinical biomarkers of disease onset and progression are thus also urgently needed to support the development of the new therapeutic agents and novel preventive and curative strategies. Effective translation from pre-clinical to clinical studies will further require extensive knowledge regarding drug activity, bioavailability and efficacy in both the pre-clinical and clinical setting, and proof of biological activity in the target tissue. During the last decades, the development of new therapeutic molecules, advance neuroimaging tools, patient derived induced stem cells and new precision medicine approaches to study ALS has significantly improved our understanding of disease. In particular, new genetic tools, neuroimaging methods, cellular probes, biomarker study and molecular techniques that achieve high spatiotemporal resolution have revealed new details about the disease onset and its progression. In our effort to provide the interested reader, clinician and researchers a comprehensive summaries and new findings in this field of ALS research, hereby we have created this electronic book which comprises of twenty seven chapters having various reviews, perspective and original research articles. All these chapters and articles in this book not only summarize the cutting-edge techniques, approaches, cell and animal models to study ALS but also provide unprecedented coverage of the current developments and new hypothesis emerging in ALS research. Some examples are novel genetic and cell culture based models, mitochondria-mediated therapy, oxidative stress and ROS mechanism, development of stem cells and mechanism-based therapies as well as novel biomarkers for designing and testing effective therapeutic strategies that can benefit ALS patients who are at the earlier stages in the disease. I am extremely grateful to all the contributors to this book and want to thank them for their phenomenal efforts.

Mitochondria --- neurodegenerative disease --- Ca2+ signaling --- multidrug therapy --- Amyotrophic lateral sclerosis (ALS) --- Superoxide dismutase 1 (SOD1) --- Motor neuron disease (MND) --- excitotoxicity --- Riluzole --- multifactorial disease --- Mitochondria --- neurodegenerative disease --- Ca2+ signaling --- multidrug therapy --- Amyotrophic lateral sclerosis (ALS) --- Superoxide dismutase 1 (SOD1) --- Motor neuron disease (MND) --- excitotoxicity --- Riluzole --- multifactorial disease

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This Special Issue entitled “β-glucan in foods and health benefits” reports on the health benefits of indigestible carbohydrates with respect to metabolic diseases and immune functions. The effects of β-glucan have been investigated through the use isolated preparations or natural dietary fibers from whole grain cereals and brans, yeasts, or Euglena. This Special Issue includes original research articles that are based on human intervention studies that address the effects of β-glucan on metabolic diseases and immune function-related markers as well as in vitro and in vivo studies. It also reviews the health benefits of β-glucans in humans.

Research & information: general --- Biology, life sciences --- Food & society --- humans --- oat β-glucan --- acute glycemic response --- dietary fiber --- preload --- carbohydrates --- β-1,3-glucan --- Euglena gracilis --- Ca2+ signaling --- intestinal epithelial cell --- intravital imaging --- small intestine --- immune system --- barley --- β-glucan --- microarray --- short chain fatty acids --- lipid metabolism. --- low molecular weight --- fermentation --- prebiotics --- Autreobasidium pullulans --- β-1,3-1,6-glucan --- physiological function --- oat beta-glucan --- colitis --- Crohn's disease --- apoptosis --- autophagy --- TLRs --- Dectin-1 --- rats --- L cell --- glucagon-like peptide 1 (GLP-1) --- glucose tolerance --- short-chain fatty acids --- sIgA --- microbiota --- randomized clinical trial --- symptoms --- gastrointestinal tract --- musculo-skeletal system --- oats --- oatmeal --- beta-glucan --- beta glucan --- health claim --- regulation --- food-health relationship --- gastritis --- inflammatory process --- antioxidant properties --- paramylon --- abdominal fat --- DNA microarray --- gene ontology --- PPAR signaling --- humans --- oat β-glucan --- acute glycemic response --- dietary fiber --- preload --- carbohydrates --- β-1,3-glucan --- Euglena gracilis --- Ca2+ signaling --- intestinal epithelial cell --- intravital imaging --- small intestine --- immune system --- barley --- β-glucan --- microarray --- short chain fatty acids --- lipid metabolism. --- low molecular weight --- fermentation --- prebiotics --- Autreobasidium pullulans --- β-1,3-1,6-glucan --- physiological function --- oat beta-glucan --- colitis --- Crohn's disease --- apoptosis --- autophagy --- TLRs --- Dectin-1 --- rats --- L cell --- glucagon-like peptide 1 (GLP-1) --- glucose tolerance --- short-chain fatty acids --- sIgA --- microbiota --- randomized clinical trial --- symptoms --- gastrointestinal tract --- musculo-skeletal system --- oats --- oatmeal --- beta-glucan --- beta glucan --- health claim --- regulation --- food-health relationship --- gastritis --- inflammatory process --- antioxidant properties --- paramylon --- abdominal fat --- DNA microarray --- gene ontology --- PPAR signaling

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This Special Issue entitled “β-glucan in foods and health benefits” reports on the health benefits of indigestible carbohydrates with respect to metabolic diseases and immune functions. The effects of β-glucan have been investigated through the use isolated preparations or natural dietary fibers from whole grain cereals and brans, yeasts, or Euglena. This Special Issue includes original research articles that are based on human intervention studies that address the effects of β-glucan on metabolic diseases and immune function-related markers as well as in vitro and in vivo studies. It also reviews the health benefits of β-glucans in humans.

Research & information: general --- Biology, life sciences --- Food & society --- humans --- oat β-glucan --- acute glycemic response --- dietary fiber --- preload --- carbohydrates --- β-1,3-glucan --- Euglena gracilis --- Ca2+ signaling --- intestinal epithelial cell --- intravital imaging --- small intestine --- immune system --- barley --- β-glucan --- microarray --- short chain fatty acids --- lipid metabolism. --- low molecular weight --- fermentation --- prebiotics --- Autreobasidium pullulans --- β-1,3-1,6-glucan --- physiological function --- oat beta-glucan --- colitis --- Crohn’s disease --- apoptosis --- autophagy --- TLRs --- Dectin-1 --- rats --- L cell --- glucagon-like peptide 1 (GLP-1) --- glucose tolerance --- short-chain fatty acids --- sIgA --- microbiota --- randomized clinical trial --- symptoms --- gastrointestinal tract --- musculo-skeletal system --- oats --- oatmeal --- beta-glucan --- beta glucan --- health claim --- regulation --- food-health relationship --- gastritis --- inflammatory process --- antioxidant properties --- paramylon --- abdominal fat --- DNA microarray --- gene ontology --- PPAR signaling --- n/a --- Crohn's disease

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Mitochondrial transporters are membrane-inserted proteins which provide a link between metabolic reactions occurring within the mitochondrial matrix and outside the organelles by catalyzing the translocation of numerous solutes across the mitochondrial membrane. They include the mitochondrial carrier family members, the proteins involved in pyruvate transport, ABC transporters and channels, and are, therefore, essential for many biological processes and cell homeostasis. Identification and functional studies of many mitochondrial transporters have been performed over the years using both in vitro and in vivo systems. The few recently solved structures of these transporters have paved the way for further investigations. Furthermore, alterations in their function are responsible for several diseases.

Research & information: general --- Biology, life sciences --- hypoxia --- resistance to hypoxia --- mitochondria --- mitochondrial calcium transport --- mitochondrial calcium uniporter complex --- mitochondrial Ca2+-induced permeability transition pore --- cyclophilin D --- ATP synthase --- disease --- error of metabolism --- mitochondrial carrier --- mitochondrial carrier disease --- mitochondrial disease --- mitochondrial transporter --- membrane transport --- mutation --- SLC25. --- mitochondrial permeability transition --- apoptosis --- necrosis --- ischemia/reperfusion --- cancer --- neurodegeneration --- cyclosporin A --- metabolite transport --- mitochondrial pyruvate carrier --- sideroflexin --- TOM --- TIM chaperones --- TIM22 --- protein translocation --- mitochondrial biogenesis --- amino acid --- biological function --- ion --- inner mitochondrial membrane --- mitochondrial carrier family --- organic acid --- substrate specificity --- transport mechanism --- vitamin --- USMG5/DAPIT --- glucose-stimulated insulin secretion --- glucose-induced expression --- membrane subunits of ATP synthase --- ATP synthase oligomers mitochondrial cristae morphology --- metabolism --- metabolic disorders --- adult-onset type II citrullinemia (CTLN2) --- aspartate/glutamate carrier (AGC) --- animal model --- argininosuccinate synthetase (ASS) --- aversion to carbohydrates --- citrin --- food taste --- neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) --- protein–protein interaction --- MPC --- lifespan --- pyruvate metabolism --- mitochondrial transport --- peroxisomes --- carrier --- cofactor --- ABC transporter --- aquaporin --- ion channels --- potassium channels --- ATP --- calcium --- ROS --- potassium channel openers --- MCF --- function --- plant metabolism --- plant development --- diseases --- VDAC1 --- virus --- pancreatic islets --- β-cell --- diabetes --- glucotoxicity --- glucolipotoxicity --- lipotoxicity --- mitochondrial carriers --- SLC transporters --- SLC25 --- SLC54 --- SLC55 --- LETM --- SLC56 --- sequence analysis --- protein targeting --- Voltage-Dependent Anion selective Channel --- isoforms --- oxidative post-translational modification --- gene promoter --- yeast --- bioenergetics --- SLC25A1 --- CIC --- CTP --- citrate --- inflammation --- 22.q11.2 --- NAFLD/NASH --- carnitine --- carnitine acyl-carnitine carrier --- carnitine acyl-carnitine translocase --- post-translational modification --- solute carrier family 25 --- SLC25A20 --- MCU --- mitochondrial Ca2+ uniporter --- Ca2+ signaling --- mitochondrial metabolism --- skeletal muscle mitochondria --- SLC25A51 --- NAD+ transporters --- NAD --- electrophysiology --- ATP-dependent potassium channel