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Endoplasmic Reticulum Mitochondria Associations And Amyotrophic Lateral Sclerosis
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Book Synopsis Endoplasmic Reticulum-mitochondria Associations and Amyotrophic Lateral Sclerosis by : Radu Stoica
Download or read book Endoplasmic Reticulum-mitochondria Associations and Amyotrophic Lateral Sclerosis written by Radu Stoica and published by . This book was released on 2013 with total page 434 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Mitochondrial Dysfunction by : Lawrence H. Lash
Download or read book Mitochondrial Dysfunction written by Lawrence H. Lash and published by Elsevier. This book was released on 2013-10-22 with total page 527 pages. Available in PDF, EPUB and Kindle. Book excerpt: Methods in Toxicology, Volume 2: Mitochondrial Dysfunction provides a source of methods, techniques, and experimental approaches for studying the role of abnormal mitochondrial function in cell injury. The book discusses the methods for the preparation and basic functional assessment of mitochondria from liver, kidney, muscle, and brain; the methods for assessing mitochondrial dysfunction in vivo and in intact organs; and the structural aspects of mitochondrial dysfunction are addressed. The text also describes chemical detoxification and metabolism as well as specific metabolic reactions that are especially important targets or indicators of damage. The methods for measurement of alterations in fatty acid and phospholipid metabolism and for the analysis and manipulation of oxidative injury and antioxidant systems are also considered. The book further tackles additional methods on mitochondrial energetics and transport processes; approaches for assessing impaired function of mitochondria; and genetic and developmental aspects of mitochondrial disease and toxicology. The text also looks into mitochondrial DNA synthesis, covalent binding to mitochondrial DNA, DNA repair, and mitochondrial dysfunction in the context of developing individuals and cellular differentiation. Microbiologists, toxicologists, biochemists, and molecular pharmacologists will find the book invaluable.
Book Synopsis The Role of Mitochondria, Oxidative Stress and Altered Calcium Homeostasis in Amyotrophic Lateral Sclerosis: From Current Developments in the Laboratory to Clinical Treatments by : Manoj Kumar Jaiswal
Download or read book The Role of Mitochondria, Oxidative Stress and Altered Calcium Homeostasis in Amyotrophic Lateral Sclerosis: From Current Developments in the Laboratory to Clinical Treatments written by Manoj Kumar Jaiswal and published by Frontiers Media SA. This book was released on 2017-04-17 with total page 338 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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. Manoj Kumar Jaiswal, Ph.D. February 5, 2017 New York, NY
Book Synopsis The Role of ER-mitochondria Contact Sites in the Regulation of Glucose Metabolism by : Kirstin Arianna Tamucci
Download or read book The Role of ER-mitochondria Contact Sites in the Regulation of Glucose Metabolism written by Kirstin Arianna Tamucci and published by . This book was released on 2023 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: The mechanisms by which mitochondria convert nutrients into cellular energy have been described in intricate detail, and yet, the regulation and compartmentalization of such metabolic pathways are poorly understood. As a result, the underlying causes of mitochondrial dysfunction and bioenergetic deficiency in diseases such as amyotrophic lateral sclerosis (ALS) remain elusive. To address this longstanding gap in the field, we first sought to understand how the metabolism of glucose and glucose-derived pyruvate are regulated in the cell. Previous research has suggested that this metabolic regulation is mediated by specialized lipid raft domains of the endoplasmic reticulum (ER) in close contact with mitochondria, referred to as mitochondria-associated ER membranes (MAM). Using density gradient ultracentrifugation and immunoblotting techniques, we found that MAM domains play a role in the compartmentalization of glycolysis by recruiting and promoting the interaction of specific glycolytic enzymes.
Book Synopsis Investigating ER-mitochondria Signalling in Amyotrophic Lateral Sclerosis by : Naomi Hartopp
Download or read book Investigating ER-mitochondria Signalling in Amyotrophic Lateral Sclerosis written by Naomi Hartopp and published by . This book was released on 2020 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis The Role of Mitochondria, Oxidative Stress and Altered Calcium Homeostasis in Amyotrophic Lateral Sclerosis: From Current Developments in the Laboratory to Clinical Treatments by :
Download or read book The Role of Mitochondria, Oxidative Stress and Altered Calcium Homeostasis in Amyotrophic Lateral Sclerosis: From Current Developments in the Laboratory to Clinical Treatments written by and published by . This book was released on 2017 with total page 0 pages. Available in PDF, EPUB and Kindle. Book excerpt: 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.
Book Synopsis The Central Role of Calcium Dysregulation in a Primary Cell Culture Model of Amyotrophic Lateral Sclerosis by : Miranda Tradewell
Download or read book The Central Role of Calcium Dysregulation in a Primary Cell Culture Model of Amyotrophic Lateral Sclerosis written by Miranda Tradewell and published by . This book was released on 2008 with total page 247 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Endoplasmic Reticulum by : A. Benedetti
Download or read book Endoplasmic Reticulum written by A. Benedetti and published by IOS Press. This book was released on 2005 with total page 172 pages. Available in PDF, EPUB and Kindle. Book excerpt: The endoplasmic reticulum is a continuous membrane network in the cytosol, which encloses its internal compartment, the endoplasmic reticulum lumen. Several metabolic pathways are compartmentalised within the ER lumen, for example hydrolysis of glucose 6-phosphate, glucuronidation of endo- xenobiotics, posttranslational modification of proteins including redox reactions required for oxidative folding, oxidoreduction of steroid hormones, synthesis of ascorbate. Therefore, enzyme activities of these pathways depend on the special luminal microenvironment, on access to substrates and on release of products. However, in spite of great efforts, the molecular mechanism for the generation and maintenance of this special microenvironment still remains to be elucidated. Beside the well-known functions of the endoplasmic reticulum, such as calcium signaling and the synthesis of secretory proteins, recent findings underlined the importance of the intraluminal redox biochemistry and the role of membrane transporters. The field is currently undergoing extensive reappraisal, new transporters have been identified either molecular or functional level.The local synthesis and the membrane transport of redox active compounds (pro- and antioxidants) seem to be important not only in the disulfide bond formation, but also in the generation of intracellular proliferative/apoptotic signals. The different points of views in this publication help highlight the potential importance of several recently described phenomena, whose significance needs elucidation.
Book Synopsis The ER Mitochondria Calcium Cycle in Mouse G93A HSOD1 Motor Neurons - Identification of Molecular Targets for the Development of New Treatment Strategies for Amyotrophic Lateral Sclerosis by :
Download or read book The ER Mitochondria Calcium Cycle in Mouse G93A HSOD1 Motor Neurons - Identification of Molecular Targets for the Development of New Treatment Strategies for Amyotrophic Lateral Sclerosis written by and published by . This book was released on 2014 with total page 208 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis The ER Mitochondria Calcium Cycle in Mouse G93A HSOD1 Motor Neurons by : Janin Lautenschläger
Download or read book The ER Mitochondria Calcium Cycle in Mouse G93A HSOD1 Motor Neurons written by Janin Lautenschläger and published by . This book was released on 2014 with total page 208 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Uncovering the Role of Misfolded SOD1 in the Pathogenesis of Amyotrophic Lateral Sclerosis by : Sarah Pickles
Download or read book Uncovering the Role of Misfolded SOD1 in the Pathogenesis of Amyotrophic Lateral Sclerosis written by Sarah Pickles and published by . This book was released on 2015 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Organelle Contact Sites by : Mitsuo Tagaya
Download or read book Organelle Contact Sites written by Mitsuo Tagaya and published by Springer. This book was released on 2017-08-16 with total page 254 pages. Available in PDF, EPUB and Kindle. Book excerpt: This book provides the first comprehensive coverage of the quickly evolving research field of membrane contact sites (MCS). A total of 16 chapters explain their organization and role and unveil the significance of MCS for various diseases. MCS, the intracellular structures where organellar membranes come in close contact with one another, mediate the exchange of proteins, lipids, and ions. Via these functions, MCS are critical for the survival and the growth of the cell. Owing to that central role in the functioning of cells, MCS dysfunctions lead to important defects of human physiology, influence viral and bacterial infection, and cause disease such as inflammation, type II diabetes, neurodegenerative disorders, and cancer. To approach such a multifaceted topic, this volume assembles a series of chapters dealing with the full array of research about MCS and their respective roles for diseases. Most chapters also introduce the history and the state of the art of MCS research, which will initiate discussion points for the respective types of MCS for years to come. This work will appeal to all cell biologists as well as researchers on diseases that are impacted by MCS dysfunction. Additionally, it will stimulate graduate students and postdocs who will energize, drive, and develop the research field in the near future.
Book Synopsis Investigating the Effect of Amyotrophic Lateral Sclerosis-associated Mutant Vesicle-associated Membrane Protein-associated Protein B on Axonal Transport by :
Download or read book Investigating the Effect of Amyotrophic Lateral Sclerosis-associated Mutant Vesicle-associated Membrane Protein-associated Protein B on Axonal Transport written by and published by . This book was released on 2012 with total page 238 pages. Available in PDF, EPUB and Kindle. Book excerpt: Thus VAPBP56Smay nsport of mitochondria by disrupting Ca2+ homeostasis and affecting the interaction of Mirol/kinesin-1 with tubulin. Publications in refereed journals arising from the thesis Morotz GM, De Vos KJ, Vagnoni A, Ackerley S, Shaw CE, Miller CC. Amyotrophic lateral sclerosis-associated mutant VAPBP56S perturbs calcium homeostasis to disrupt axonal transport of mitochondria. Hum. Mol. Genet. (2012) 21:1979-1988. De Vos KJ, Morotz GM, Stoica R, Tudor EL, Lau KF, Ackerley S, Warley A, Shaw CE, Miller CC. VAPB interacts with the mitochondrial protein PTPIP51 to regulate calcium homeostasis. Hum. Mol. Genet. (2012) 21:1299-1311.
Book Synopsis Understanding Neuromuscular Health and Disease: Advances in Genetics, Omics, and Molecular Function by : William Duddy
Download or read book Understanding Neuromuscular Health and Disease: Advances in Genetics, Omics, and Molecular Function written by William Duddy and published by . This book was released on 2021 with total page 318 pages. Available in PDF, EPUB and Kindle. Book excerpt: This compilation focuses on recent advances in the molecular and cellular understandingof neuromuscular biology, and the treatment of neuromuscular disease.These advances are at the forefront of modern molecular methodologies, oftenintegrating across wet-lab cell and tissue models, dry-lab computational approaches,and clinical studies. The continuing development and application ofmultiomics methods offer particular challenges and opportunities in the field,not least in the potential for personalized medicine.
Book Synopsis Molecular Links Between Mitochondrial Damage and Parkinson's Disease and Related Disorders by : Yuzuru Imai
Download or read book Molecular Links Between Mitochondrial Damage and Parkinson's Disease and Related Disorders written by Yuzuru Imai and published by Frontiers Media SA. This book was released on 2021-09-28 with total page 165 pages. Available in PDF, EPUB and Kindle. Book excerpt:
Book Synopsis Amyotrophic Lateral Sclerosis by : Muralidhar L. Hegde
Download or read book Amyotrophic Lateral Sclerosis written by Muralidhar L. Hegde and published by BoD – Books on Demand. This book was released on 2020-08-26 with total page 162 pages. Available in PDF, EPUB and Kindle. Book excerpt: A flurry of recent research on the role of the RNA/DNA-binding proteins TDP-43 and FUS as well as a dozen other factors (e.g., C9ORF72 and profilin) has led to a new paradigm in our understanding of the pathobiology of the motor neuron disease, Amyotrophic Lateral Sclerosis (ALS). How these factors trigger neuromuscular dysfunction is critical for developing more effective ALS therapeutics. The ‘gain-of-toxicity’ or ‘loss-of-function’ of these etiological factors is a key question. Recent studies on the imbalance in genome damage versus repair have opened avenues for potential DNA repair-based therapeutics. This book highlights emerging science in the area of ALS and discusses key approaches and mechanisms essential for developing a cure for ALS.
Book Synopsis Neuropathology of Neurodegenerative Diseases by : Gabor G. Kovacs
Download or read book Neuropathology of Neurodegenerative Diseases written by Gabor G. Kovacs and published by Cambridge University Press. This book was released on 2017-12-13 with total page 320 pages. Available in PDF, EPUB and Kindle. Book excerpt: This practical guide to the diagnosis of neurodegenerative diseases discusses modern molecular techniques, morphological classification, fundamentals of clinical symptomology, diagnostic pitfalls and immunostaining protocols. It is based on the proteinopathy concept of neurodegenerative disease, which has influenced classification and provides new strategies for therapy. Numerous high-quality images, including histopathology photomicrographs and neuroradiology scans, accompany the description of morphologic alterations and interpretation of immunoreactivities. Diagnostic methods and criteria are placed within recent developments in neuropathology, including the now widespread application of immunohistochemistry. To aid daily practice, the guide includes diagnostic algorithms and offers personal insights from experienced experts in the field. Special focus is given to the way brain tissue should be handled during diagnosis. This is a must-have reference for medical specialists and specialist medical trainees in the fields of pathology, neuropathology and neurology working with neuropathologic features of neurodegenerative diseases.