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It has become evident over the last years that abnormalities in RNA processing play a fundamental part in the pathogenesis of neurodegenerative diseases. Cellular viability depends on proper regulation of RNA metabolism and subsequent protein synthesis, which requires the interplay of many processes including transcription, pre---mRNA splicing, mRNA editing as well as mRNA stability, transport and translation. Dysfunction in any of these processes, often caused by mutations in the coding and non--- coding RNAs, can be very destructive to the cellular environment and consequently impair neural viability. The result of this RNA toxicity can lead to a toxic gain of function or a loss of function, depending on the nature of the mutation. For example, in repeat expansion disorders, such as the newly discovered hexanucleotide repeat expansion in theC9orf72 gene found in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), a toxic gain of function leads to the formation of RNA foci and the sequestration of RNA binding proteins (RBPs). This in return leads to a loss of function of those RBPs, which is hypothesized to play a significant part in the disease progression of ALS and FTD. Other toxicities arising from repeat expansions are the formation of RNA foci, bi---directional transcription and production of repeat associated non---ATG (RAN) translation products.This book will touch upon most of these disease mechanisms triggered by aberrant RNA metabolism and will therefore provide a broad perspective of the role of RNA processing and its dysfunction in a variety of neurodegenerative disorders, including ALS, FTD, Alzheimer's disease, Huntington's disease, spinal muscular atrophy, myotonic dystrophy and ataxias. The proposed authors are leading scientists in the field and are expected to not only discuss their own work, but to be inclusive of historic as well as late breaking discoveries. The compiled chapters will therefore provide a unique collection of novel studies and hypotheses aimed to describe the consequences of altered RNA processing events and its newest molecular players and pathways.

The book reviews clinical trial methodology as it pertains to drug development in psychiatry. The reader will understand the process of drug development in psychiatry from discovery through marketing with the help of clinically relevant examples. The reader will appreciate the history of drug development in psychiatry dating back to the era of serendipitous discovery and culminating in an era of new and highly focused targets. Readers will understand how drug development in psychiatry has changed and adapted with the discovery of novel mechanism of action drugs. Novel drugs and disease targets have changed the way developers and regulatory agencies think about clinical trial methodology.The book elucidates how biomarkers, genetics and advances in neuroscience and neuroimaging have influenced drug development approaches, which will ultimately change the practice of psychiatry. The book will be broken down into the following sections:a. Prior to the 1960s - Drug discovery by chance observationb. The last 50 years ¿ refined targeting of CNS drugs without the discovery of mechanistically new drugsc. The future ¿ the discovery and development of mechanistically new drugs. The examination of new targets, genetics and biomarkers.

This new edition provides comprehensive coverage of the variety and complexity of the roles that glycoconjugates play in the cells of the nervous system. Basic fundamental principles as well as the latest developments in neural glycobiology are discussed.  Topics covered range from the structure and metabolism of the saccharide chains and current approaches used in their study, to changes glycoconjugates undergo during development and aging of the nervous system and the roles they have in neurological disease. New topics include a detailed discussion of cells found within the nervous system, an extensive listing of congenital disorders of glycosylation of both proteins and lipids, the roles of glycans in neuronal axon growth/guidance and voltage-gated channels, the role of intra-lysosomal luminal vesicles in lysosomal storage disorders, and, in the time of the COVID-19 pandemic, the role of carbohydrates in infection by SARS-CoV-2. The breadth and depth of topics covered make this an essential reference for those new to the field as well as for more experienced investigators.

This book aims to provide a comprehensive and timely review of new findings in motoneuron research. Recent findings have revealed that motoneurons are more complex and have more extensive functions than previously imagined. Some of the molecular and genetic pathways that orchestrate the development of motoneurons have been discovered, as have the mechanisms responsible for the selective innervation of muscles by specific pools of motoneurons. These novel findings are revolutionizing ideas about the function of motoneurons and have important implications for motoneuron disease.  Chapters from several of the foremost figures in the field are included in this book and will emphasize how basic science is the engine driving the discovery of novel treatments for degenerative motoneuron diseases. Cutting edge developments in the use of pluripotent stem cells to correct motoneuron disease will be also be covered.  This book would be useful to students of basic motoneuron physiology, locomotor behavior and motor control. This book would also be of interest to professional neuroscientists, neurologists, and neurosurgeons.

Modern neuroscience has presented new opportunities for exploring the molecular and neural mechanisms controlling specific social responses. This book reviews insights into the neural circuits underlying a particularly fascinating form of social interaction, parental behavior. This book presents a detailed review of maternal and paternal behavior of particular mammalian species. It offer neuroscientists a spectrum of specific mammals that can be used as experimental models to explore particular topics on the functions of the nervous system. It shows that results coming from the laboratory can be translated into useful information for raising mammals on the farm, and it stimulates biologists to gain insights into the underpinnings of the complex mechanisms governing mammalian behavior in the wild.  It also discusses the implications of this research for human parental behavior. 

This book aims to provide a state-of-the-art summary of what is currently known about brain glycogen metabolism, detailing the recent advances in our understanding of why glycogen is so critical for normal brain function. The role of glycogen in cellular neurophysiology remains largely unclear and its specific contribution to the energy demand of brain cells is still elusive.Glycogen is the sole cerebral glucose reserve and is emerging as a fundamental component of brain energy metabolism. Pharmacological or genetic manipulation of glycogen metabolism in the brain impairs memory formation and increases susceptibility to epileptic seizures and cortical spreading depression. Glycogen is also directly implicated in abnormal neuronal excitability and mental retardation that characterize brain disorders like Lafora disease and Pompe disease.  

This book contains a compendium of induced pluripotent stem cells (iPSCs) articles and reviews concerning state of the art technologies and how they are being applied to human neurodevelopmental disorders.

The book focuses on implications of traditional and processed foods for autism spectrum disorder (ASD) intervention and management. The neuroprotective effects of various fruits, vegetables, nuts and seeds phytochemicals, and other natural bioactive ingredients against ASD and related conditions are discussed.

The book focuses on implications of traditional and processed foods for autism spectrum disorder (ASD) intervention and management. The neuroprotective effects of various fruits, vegetables, nuts and seeds phytochemicals, and other natural bioactive ingredients against ASD and related conditions are discussed.

Systems biology is an inter-disciplinary field that focuses on complex interactions within biological systems to reveal 'emergent properties' - properties of cells and groups of cells functioning as a system whose actual and theoretical description is only possible using Systems Biology techniques.

From the preface: ¿Neural Metabolism In Vivo aims to provide a comprehensive overview of neurobiology by presenting the basic principles of up-to-date and cutting-edge technology, as well as their application in assessing the functional, morphological and metabolic aspects of the brain.  Investigation of neural activity of the living brain via neurovascular coupling using multimodal imaging techniques extended our understanding of fundamental neurophysiological mechanisms, regulation of cerebral blood flow in connection to neural activity and the interplay between neurons, astrocytes and blood vessels.  Constant delivery of glucose and oxygen for energy metabolism is vital for brain function, and the physiological basis of neural activity can be assessed through measurements of cerebral blood flow and consumption of glucose and oxygen¿. This book presents the complex physiological and neurochemical processes of neural metabolism and function in response to various physiological conditions and pharmacological stimulations.  Neurochemical detection technologies and quantitative aspects of monitoring cerebral energy substrates and other metabolites in the living brain are described under the ¿Cerebral metabolism of antioxidants, osmolytes and others in vivö section.  Altogether, the advent of new in vivo tools has transformed neuroscience and neurobiology research, and demands interdisciplinary approaches as each technology could only approximate a very small fraction of the true complexity of the underlying biological processes.  However, translational values of the emerging in vivo methods to the application of preclinical to clinical studies cannot be emphasized enough.  Thus, it is our hope that advances in our understanding of biochemical, molecular, functional and physiological processes of the brain could eventually help people with neurological problems, which are still dominated by theunknowns.¿ -- In-Young Choi and Rolf Gruetter

This is the first book to assemble the leading researchers in the field of LRRK2 biology and neurology and provide a snapshot of the current state of knowledge, encompassing all major aspects of its function and dysfunction.

The diverse array of bioactive nutrients present in these natural products plays a pivotal role in prevention and cure of various neurodegenerative diseases, disorders, or insults, such as Alzheimer's Disease, Parkinson's Disease, Huntington's Disease, traumatic brain injury, and other neuronal dysfunctions.

Fundamental biochemical studies of basic brain metabolism focusing on the neuroactive amino acids glutamate and GABA combined with the seminal observation that one of the key enzymes, glutamine synthetase is localized in astroglial cells but not in neurons resulted in the formulation of the term "The Glutamate-Glutamine Cycle." In this cycle glutamate released from neurons is taken up by surrounding astrocytes, amidated by the action of glutamine synthetase to glutamine which can be transferred back to the neurons. The conversion of glutamate to glutamine is like a stealth technology, hiding the glutamate molecule which would be highly toxic to neurons due to its excitotoxic action. This series of reactions require the concerted and precise interaction of a number of enzymes and plasma membrane transporters, and this volume provides in-depth descriptions of these processes. Obviously such a series of complicated reactions may well be prone to malfunction and therefore neurological diseases are likely to be associated with such malfunction of the enzymes and transporters involved in the cycle. These aspects are also discussed in several chapters of the book.A number of leading experts in neuroscience including intermediary metabolism, enzymology and transporter physiology have contributed to this book which provides comprehensive discussions of these different aspects of the functional importance of the glutamate-glutamine cycle coupling homeostasis of glutamatergic, excitatory neurotransmission to basic aspects of brain energy metabolism. This book will be of particular importance for students as well as professionals interested in these fundamental processes involved in brain function and dysfunction.

Provides a timely overview of critical advances in molecular and cellular neurobiology, covers key methodologies driving progress, and highlights key future directions for research on neuronal injury and neurodegeneration relevant to neuronal brain pathologies.

It has become evident over the last years that abnormalities in RNA processing play a fundamental part in the pathogenesis of neurodegenerative diseases. Cellular viability depends on proper regulation of RNA metabolism and subsequent protein synthesis, which requires the interplay of many processes including transcription, pre---mRNA splicing, mRNA editing as well as mRNA stability, transport and translation. Dysfunction in any of these processes, often caused by mutations in the coding and non--- coding RNAs, can be very destructive to the cellular environment and consequently impair neural viability. The result of this RNA toxicity can lead to a toxic gain of function or a loss of function, depending on the nature of the mutation. For example, in repeat expansion disorders, such as the newly discovered hexanucleotide repeat expansion in theC9orf72 gene found in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), a toxic gain of function leads to the formation of RNA foci and the sequestration of RNA binding proteins (RBPs). This in return leads to a loss of function of those RBPs, which is hypothesized to play a significant part in the disease progression of ALS and FTD. Other toxicities arising from repeat expansions are the formation of RNA foci, bi---directional transcription and production of repeat associated non---ATG (RAN) translation products.This book will touch upon most of these disease mechanisms triggered by aberrant RNA metabolism and will therefore provide a broad perspective of the role of RNA processing and its dysfunction in a variety of neurodegenerative disorders, including ALS, FTD, Alzheimer's disease, Huntington's disease, spinal muscular atrophy, myotonic dystrophy and ataxias. The proposed authors are leading scientists in the field and are expected to not only discuss their own work, but to be inclusive of historic as well as late breaking discoveries. The compiled chapters will therefore provide a unique collection of novel studies and hypotheses aimed to describe the consequences of altered RNA processing events and its newest molecular players and pathways.

This monograph begins with a general description of the cytoskeleton in axonal development and pathology and then moves to more detailed descriptions of particular components, including microtubules and associated proteins, neurofilaments and interacting proteins, actin and its binding proteins, and glial fibrillary acidic protein.

Provides a timely overview of critical advances in molecular and cellular neurobiology, covers key methodologies driving progress, and highlights key future directions for research on neuronal injury and neurodegeneration relevant to neuronal brain pathologies.

This newest volume of Advances in Neurobiology discusses the utilization of genomic and proteomic technologies, to address facets of neurobiology including development and epigenetic regulation, functions in learning and memory, and changes associated with neurological and psychiatric disorders.

This book contains a compendium of induced pluripotent stem cells (iPSCs) articles and reviews concerning state of the art technologies and how they are being applied to human neurodevelopmental disorders.

This book aims to provide a state-of-the-art summary of what is currently known about brain glycogen metabolism, detailing the recent advances in our understanding of why glycogen is so critical for normal brain function.