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Mitochondria In Neurological Disorders

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Mitochondria in Neurological Disorders

Mitochondria in Neurological Disorders Book
Author : Rajat Sandhir
Publisher : Academic Press
Release : 2022-06-15
ISBN : 9780128217313
Language : En, Es, Fr & De

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Book Description :

Mitochondria in Neurological Diseases highlights various therapeutic approaches targeting mitochondria in neurological conditions. Focused on diverse neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, Huntington's disease, depression, and Amyotrophic lateral sclerosis, topics span basic mitochondrial physiology to mitochondrial dynamics and to the altered states of the nervous system. This book is appropriate for anyone interested in learning more about the physiological and pathophysiological functions of mitochondria in the nervous system. Provides up-to-date information on role of mitochondria in nervous system for graduate students and researchers Presents chapters on energy substrate utilization, calcium handling, mitochondria-organelle communication, and cell signaling Discusses recent techniques in understanding mitochondrial functions in the nervous system Emphasizes the role of altered mitochondrial functions in various neurological conditions and their potential treatment strategies for targeting mitochondria Includes chapter references (key articles, books, protocols) for additional detailed study

Mitochondria and Brain Disorders

Mitochondria and Brain Disorders Book
Author : Stavros Baloyannis
Publisher : BoD – Books on Demand
Release : 2020-03-11
ISBN : 1789855314
Language : En, Es, Fr & De

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Book Description :

The mitochondrion is a unique and ubiquitous organelle that contains its own genome, encoding essential proteins that are major components of the respiratory chain and energy production system. Mitochondria play a dominant role in the life and function of eukaryotic cells including neurons and glia, as their survival and activity depend upon mitochondrial energy production and supply. Besides energy production, mitochondria also play a vital role in calcium homeostasis and may induce apoptosis by excitotoxicity. Mitochondrial dysfunction is related to common neurological diseases, such as Parkinson's disease, Alzheimer's disease, Friedreich's ataxia, Huntington's disease, and Multiple Sclerosis. An efficient treatment of mitochondrial dysfunction would open new horizons in the therapeutic perspectives of a substantial number of inflammatory and degenerative neurological disorders.

Mitochondrial Disorders in Neurology

Mitochondrial Disorders in Neurology Book
Author : Anthony H.V. Schapira,S. Dimauro
Publisher : Butterworth-Heinemann
Release : 2013-10-22
ISBN : 1483193691
Language : En, Es, Fr & De

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Book Description :

Mitochondrial Disorders in Neurology provides an overview of mitochondrial diseases. This book discusses the effects of mitochondrial dysfunction based on the relevant biochemistry and molecular genetics. The abnormal muscle and mitochondrial morphology in a variety of clinical presentations from isolated ophthalmoplegia to severe encephalopathy are also elaborated. This text likewise deliberates Leber's hereditary optic neuropathy, neurodegenerative disorders, and respiratory chain defects. Other topics covered include mitochondrial DNA and the genetics of mitochondrial disease; cytochrome oxidase deficiency; use of tissue culture in the diagnosis of mitochondrial disease; and advances in mitochondrial genetics. This publication is a good source for clinicians and students concerned with the defective mitochondrial function.

Diagnosis and Management of Mitochondrial Disorders

Diagnosis and Management of Mitochondrial Disorders Book
Author : Michelangelo Mancuso,Thomas Klopstock
Publisher : Springer
Release : 2019-03-30
ISBN : 9783030055165
Language : En, Es, Fr & De

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Book Description :

This book will help readers navigate the complexity of mitochondrial disorders, by addressing the role of mitochondrial dysfunction and the complex pathophysiological mechanisms associated with a growing number of illnesses, not only of neurological interest. Further, it provides updated concepts on genotype-phenotype correlations, clinical syndromes, diagnostic algorithms and therapies. Written by the world’s foremost mitochondrial researchers, the book comprehensively presents the state-of-the-art in mitochondrial medicine, making it of interest to a wide variety of specialists, including neurologists, geneticists, internists and biologists.

Mitochondrial Dynamics and Neurodegeneration

Mitochondrial Dynamics and Neurodegeneration Book
Author : Bingwei Lu
Publisher : Springer Science & Business Media
Release : 2011-05-16
ISBN : 9789400712911
Language : En, Es, Fr & De

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Book Description :

Mitochondria are essential organelles in eukaryotic cells that control such diverse processes as energy metabolism, calcium buffering, and cell death. Recent studies have revealed that changes in mitochondrial morphology by fission and fusion, a process known as mitochondrial dynamics, is particularly important for neuronal function and survival. Defects in this process are commonly found in neurodegenerative diseases, offering a new paradigm for investigating mechanisms of neurodegeneration. To provide researchers working on neurodegenerative diseases and mitochondria with updated information on this rapidly progressing field, we have invited experts in the field to critically review recent progresses and identify future research directions. The topics include genetics of mitochondrial dynamics, mitochondrial dynamics and bioenergetics, autophagy, apoptosis, and axonal transport, and its role in neurological diseases, including Alzheimer’s, Parkinson’s, and Huntington’s diseases.

Mitochondria dependent Local Caspase Activation and Mitofusin 2 mediated Mitophagy in Neurodegeneration

Mitochondria dependent Local Caspase Activation and Mitofusin 2 mediated Mitophagy in Neurodegeneration Book
Author : Hao Chen
Publisher : Unknown
Release : 2020
ISBN : 0987650XXX
Language : En, Es, Fr & De

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Book Description :

Mitochondria are critical regulators of neuronal physiology. They play a pivotal role in energy supply and Ca2+ buffering. Thus, mitochondria can sustain the synapse and neuronal function. In this regard, the mitochondria with an impaired capacity of bioenergetics or Ca2+ buffering will lead to synapse failure and neurodegeneration. Moreover, emerging evidence has shown that damaged mitochondria are also involved in local caspase activation, which coincides with spine pruning and neurodegeneration. However, the impacts of local caspase signaling on spine dynamics and neurodegeneration are still not clarified. To clarify them is critical for neurological disorders, in which the mitochondrial deficits are prominent and hard to be fixed. Additionally, as a pivotal mitochondrial enzyme – the F1Fo ATP synthase, dysfunction of it will lead to diseases with spinopathy. To this end, we inhibited the F1Fo ATP synthase by applying the sublethal oligomycin A to primary cultured neurons. Then, it induced the mitochondrial dysfunction and local caspase signaling. Following this, we blocked the caspase signaling through its inhibitor to determine whether it can rescue the spine elimination. In this case, we can dissect the caspase signaling out of mitochondrial energy supply and examine how much the caspase signaling attributes to spine pruning. Furthermore, we will find new ways to prevent the senescence of neurons through caspase inhibition. Moreover, mitochondrial quality control is of paramount importance for neuronal survival. Notably, mitophagy is a vital mechanism for maintenance of mitochondrial quality control. A previous study has shown that Mitofusin2 (Mfn2) plays a key role in mediating mitophagy in cardiomyocytes, which was demonstrated by decreased mitophagy in Mfn2 knockdown cardiomyocytes. In addition, lowered expression levels of Mfn2 have been repeatedly found in many neurodegenerative diseases like Alzheimer’s disease, which is characterized by the accumulation of damaged mitochondrial and mitophagosomes in neurons. In this regard, reduced Mfn2 expression may lead to compromised mitophagy and mitochondrial quality control. However, whether Mfn2 is indispensable for facilitating mitophagy has not been fully investigated. To this end, we knocked down Mfn2 in primary cultured neurons. Then, we examined the state of mitophagy. Intriguingly, we observed more mitophagosomes formation in Mfn2 knock-down neurons. It indicates the Mfn2 is not necessary for mitophagy induction. Such discrepancy may arise from the heterogeneity of different cell types. Also, it may arise from some alternative adaptor proteins, which can activate mitophagy. From previous reports, people pulled down the conjugated proteins from the outer mitochondrial membrane (OMM) of senescent and healthy neurons. After screening them by mass spectrum, they found that several OMM proteins may be the potential receptors for mitophagosome formation. Among them, Voltage-Dependent Anion Channel (VDAC) is the one most reported for mitophagy induction. Accordingly, we will further knockdown VDAC in Mfn2 knock-down neurons and examine the mitophagy states. In this case, we can determine whether VDAC can work as an Mfn2-independent alternative trigger for mitophagy in neurons. In all, both local caspases signaling and mitophagy play essential roles in neurodegeneration. Regulation of them may provide us new avenues for treating neurodegenerative diseases..

Mitochondrial Dysfunction in Neurodegenerative Disorders

Mitochondrial Dysfunction in Neurodegenerative Disorders Book
Author : Amy K. Reeve,Eve M. Simcox,Michael R. Duchen,Doug M. Turnbull
Publisher : Springer
Release : 2016-06-08
ISBN : 3319286374
Language : En, Es, Fr & De

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Book Description :

This second edition brings together up-to-date contributions from leaders in the field internationally on the various ways in which mitochondrial dysfunction contributes to the pathogenesis of neurodegenerative diseases, including Parkinson’s disease, Alzheimer’s disease and multiple sclerosis. The reader is guided through the basic functions of mitochondria and the mechanisms that lead to their dysfunction, and on to the consequences of this dysfunction for neuronal function before finishing with the modelling of these disorders and discussion of new potential therapeutic targets. Additional chapters have been added to the book to reflect advances in the field and there are many new contributors and topics, including how mitochondria are degraded and the interaction of the mitochondria with pathologically relevant proteins. Mitochondrial Dysfunction in Neurodegenerative Disorders provides an accessible, authoritative guide to this important area for neurologists; research and clinical neuroscientists; neuropathologists; and residents with an interest in clinical research.

Mitochondrial Disorders in Neurology 2

Mitochondrial Disorders in Neurology 2 Book
Author : Anthony Henry Vernon Schapira,Salvatore DiMauro
Publisher : Butterworth-Heinemann Medical
Release : 2002
ISBN : 0987650XXX
Language : En, Es, Fr & De

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Book Description :

Gain practical explanations of the science underlying mitochondrial disorders in neurology. Written by leading experts, this new Blue Book helps you recognize and manage the diseases. Includes: Sections on the role of mitochondria in neurodegenerative disease The development of animal models for mitochondrial diseases The design of treatments for patients with mitochondrial defects

Mitochondrial Disorders

Mitochondrial Disorders Book
Author : Claude Desnuelle
Publisher : Springer Science & Business Media
Release : 2013-12-01
ISBN : 2817809297
Language : En, Es, Fr & De

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Book Description :

The concept of mitochondrial diseases originated in 1962 with the description by Luft and coworkers of a patient with nonthyroidal hypermetabolism due to loose coupling of oxidation and phosphorylation in muscle mitochondria. Over the following quarter of a century, thanks to W. King Engel's "ragged-red fibres" as convenient markers for mitochondrial pathology, numerous papers described clinical, morphological, and biochemical features of "mitochondrial myopathies." In 1988 the discovery of mutations in mitochondrial DNA led to an explosive expansion of research into mitochondrial disorders. Throughout the 1990s the rapid identification of multiple mitochondrial gene defects associated with clinically diverse disorders has left practitioners puzzled about diagnosing such heterogeneous and complex syndromes. Through updated data, this book discusses now what Luft aptly called "mitochondrial medicine." In so doing, it considers the pivotal role of mitochondria in drug sensitivity, their key roles in ageing, apoptosis, and neurodegeneration along with primary mitochondrial diseases due to mutations in the nuclear genome, in the mitochondrial genome, or in the cross-talk between the two genomes.

Mitochondrial Function and Dysfunction

Mitochondrial Function and Dysfunction Book
Author : Anthony Schapira
Publisher : Elsevier
Release : 2003-01-10
ISBN : 0080489079
Language : En, Es, Fr & De

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Book Description :

Mitochondria are critical to the survival of cells, therefore, it is not surprising that abnormalities in mitochondrial function may lead to human disease. This book concentrates on the biology and pathology of mitochondria, covering some ot the important basic science features of the biology of mitochondria. It then moves on to discuss the breadth of human diseases related to mitochondrial dysfunction, including Parkinson's disease, Amyotrophic Lateral Sclerosis (ALS), and Alzheimer's disease. * Provides comprehensive coverage of basic science and clinical features of mitochondrial dysfunction * Presents detailed analysis of "hot" topics in mitochondrial function and neurodegenerative diseases * Includes outstanding list of contributing authors

Molecular Analysis of Mitochondrial DNA in Neurological Disorders

Molecular Analysis of Mitochondrial DNA in Neurological Disorders Book
Author : Denise Stephania Rath
Publisher : Unknown
Release : 1992
ISBN : 0987650XXX
Language : En, Es, Fr & De

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Book Description :

Download Molecular Analysis of Mitochondrial DNA in Neurological Disorders book written by Denise Stephania Rath, available in PDF, EPUB, and Kindle, or read full book online anywhere and anytime. Compatible with any devices.

The Functions Disease Related Dysfunctions and Therapeutic Targeting of Neuronal Mitochondria

The Functions  Disease Related Dysfunctions  and Therapeutic Targeting of Neuronal Mitochondria Book
Author : J. Marie Hardwick
Publisher : John Wiley & Sons
Release : 2015-09-21
ISBN : 1119017106
Language : En, Es, Fr & De

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Book Description :

This book presents advances in the field of neuronal mitochondria – functions, relation to therapeutics, and pharmacology. For scientists and researchers in both industry and academia, this book provides detailed discussion, examples, and approaches, to illustrate the potential of mitochondria as therapeutic targets for neuronal diseases. • Helps readers understand the regulation of mitochondrial cellular processes, such as substrate metabolism, energy production, and programmed versus sporadic cell death • Offers insights on the development of strategies for targeted therapeutic approaches and potential personalized treatments • Includes examples of mitochondrial drugs, development, and mitochondria-targeted approaches for more efficient treatment methods and further developments in the field • Covers the model systems and approaches needed for the development of new drugs for the central nervous system to provide potential modern therapeutics for neurodegenerative disorders

Diagnosis and Management of Mitochondrial Disorders

Diagnosis and Management of Mitochondrial Disorders Book
Author : Michelangelo Mancuso,Thomas Klopstock
Publisher : Springer
Release : 2019-05-03
ISBN : 3030055175
Language : En, Es, Fr & De

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Book Description :

This book will help readers navigate the complexity of mitochondrial disorders, by addressing the role of mitochondrial dysfunction and the complex pathophysiological mechanisms associated with a growing number of illnesses, not only of neurological interest. Further, it provides updated concepts on genotype-phenotype correlations, clinical syndromes, diagnostic algorithms and therapies. Written by the world’s foremost mitochondrial researchers, the book comprehensively presents the state-of-the-art in mitochondrial medicine, making it of interest to a wide variety of specialists, including neurologists, geneticists, internists and biologists.

Mutations of Mitochondrial DNA Polymerase Gamma

Mutations of Mitochondrial DNA Polymerase Gamma Book
Author : Petri T. Luoma
Publisher : Unknown
Release : 2007
ISBN : 9789529228942
Language : En, Es, Fr & De

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Book Description :

Download Mutations of Mitochondrial DNA Polymerase Gamma book written by Petri T. Luoma, available in PDF, EPUB, and Kindle, or read full book online anywhere and anytime. Compatible with any devices.

Mitochondrial Dynamics and Neurodegeneration

Mitochondrial Dynamics and Neurodegeneration Book
Author : Bingwei Lu
Publisher : Springer
Release : 2013-01-02
ISBN : 9789400712928
Language : En, Es, Fr & De

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Book Description :

Mitochondria are essential organelles in eukaryotic cells that control such diverse processes as energy metabolism, calcium buffering, and cell death. Recent studies have revealed that changes in mitochondrial morphology by fission and fusion, a process known as mitochondrial dynamics, is particularly important for neuronal function and survival. Defects in this process are commonly found in neurodegenerative diseases, offering a new paradigm for investigating mechanisms of neurodegeneration. To provide researchers working on neurodegenerative diseases and mitochondria with updated information on this rapidly progressing field, we have invited experts in the field to critically review recent progresses and identify future research directions. The topics include genetics of mitochondrial dynamics, mitochondrial dynamics and bioenergetics, autophagy, apoptosis, and axonal transport, and its role in neurological diseases, including Alzheimer’s, Parkinson’s, and Huntington’s diseases.

Molecular Basis of Neurological Disorders and Their Treatment

Molecular Basis of Neurological Disorders and Their Treatment Book
Author : J.W. Gorrod,A. Albano,E. Ferrari,S. Papa
Publisher : Springer Science & Business Media
Release : 2012-12-06
ISBN : 9401131147
Language : En, Es, Fr & De

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Book Description :

cytochemical techniques (ICC) which provide a useful adjunct to investigations by immunoblotting. A particular advantage of a cytochemical approach to the investiga tion of mitochondrial disorders is that it allows the mosaic distribution of certain of these defects to be detected, whereas the tissue homogeniza tion involved in conventional enzyme assays or immunoblotting precludes this. A further advantage of MEA or ICC is that only small amounts of tissue are needed, which is important since many of the affected patients are infants or small children. The main aim of this communica tion is to outline ways in which these techniques can be used in the diagnosis and further investigation of mitochondrial disorders. Reference will be made not only to those situations in which MEA and ICC offer advantages over standard enzyme asays and immunoblotting but also to contexts in which the reverse applies. 4. 2 MATERIALS AND METHODS Muscle biopsies for cytochemical investigation were snap-frozen using isopentane cooled to - 150°C in liquid nitrogen. Samples were stored in heat-sealed polythene packets in the vapour phase of liquid nitrogen containers. 4. 2. 1 Microphotometric enzyme assays Frozen sections 8 Jlm thick were cut using a Reichert-J ung Frigocut cryostat microtome equipped with motor-driven cutting action to maintain maximal reproducibility of section thickness. Sections were picked up on microscope slides and air-dried for 15 min at room temperature.

Mitochondria Biology

Mitochondria Biology Book
Author : Anonim
Publisher : Academic Press
Release : 2020-03-17
ISBN : 0128202297
Language : En, Es, Fr & De

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Book Description :

Methods in Cell Biology Volume 155 provides an update on the step-by-step "how-to" methods to study mitochondrial structure, function and biogenesis contained in the first two editions. As in the previous editions, biochemical, cell biological, and genetic approaches are presented along with sample results, interpretations, and pitfalls for each method. New chapters in this update include Isolation of Mitochondria and Analysis of Mitochondrial Compartments, Isolation of Mitochondria from Animal Cells and Yeast, Isolation and Characterization of Mitochondria-Associated ER Membranes, Import of Proteins into Mitochondria, Proximity Labeling Methods to Assess Protein-Protein Interactions in Yeast Mitochondria, and more. Provides a step-by-step "cookbook" presentation as written by leaders in the field Covers longstanding methods that have shaped the field Includes the newest technologies and methods

Mitochondrial Metabolism

Mitochondrial Metabolism Book
Author : Jalal Pourahmad,Mohsen Rezaei
Publisher : Elsevier
Release : 2021-08-12
ISBN : 0128224169
Language : En, Es, Fr & De

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Book Description :

Mitochondrial Metabolism: An Approach for Disease Management covers mitotherapy from three combined perspectives, Pharmacology, Toxicology and Biochemistry. After an introduction from world-renowned experts, the book's chapters cover the balancing role in reduction/oxidation mitochondria play, mitochondria as targets for therapeutics through its metabolism, mitochondrial contributions to the cell death process, mitochondrial response to environmental toxicants, the mitochondrial role in aging, the impact of calorie restrictive diets, new advances in the identification of altered mitochondria associated signaling pathways in carcinogenesis, and much more. This book provides bioscientists new horizons to realize the importance of mitochondria in present-day research on therapies dealing with mitochondria associated chronic diseases, including diabetes, cancer and neurodegenerative disorders. Details the significant role of mitochondria in chronic diseases Presents new insights on the targeting of mitochondria for therapeutic purposes Includes updated results on mitotherapy and other mitochondria-oriented therapies

The Impact of Ferritins in the Nigrostriatal System

The Impact of Ferritins in the Nigrostriatal System Book
Author : Amanda Marie Snyder
Publisher : Unknown
Release : 2009
ISBN : 0987650XXX
Language : En, Es, Fr & De

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Book Description :

Iron status is higher in the substantia nigra than in other brain regions but can fluctuate as function of diet, genetics, and disease. Because iron can influence neuronal cell death and dopamine function, the body tightly controls the supply of iron needed for tasks such as a cofactor for enzymes but also has mechanisms in place to sequester iron to limit oxidative stress. The major protein that accomplishes this latter task is ferritin, with the ferroxidase properties belonging to the H subunit. In this thesis, mice genetically altered to be heterozygous for a mutation in the H-ferritin gene were utilized to examine the impact of H-ferritin-deficiency on the dopaminergic nigrostriatal system. The distribution of mitochondrial ferritin, a protein structurally and functionally similar to Hferritin, was also explored in mouse and human brain. Using high-resolution magic angle spin proton magnetic resonance spectroscopy (HR-MAS 1H MRS), significant increases in glutamate levels were found in the striatum and ventral midbrain in H-ferritin-deficient animals as compared to wild-type. While lactate was increased in the ventral midbrain of H-ferritin-deficient animals, it was decreased in the striatum. Dopamine transporter (DAT) expression in the substantia nigra and striatum was unchanged with age or by the loss of ferritin. Overall, the neurochemical profile is suggestive of neuronal iron deficiency. Because iron deficiency causes long-term effects on the dopaminergic system and may influence the onset of or susceptibility to neurologic disorders, the number of tyrosine hydroxylase dopaminergic neurons in the substantia nigra pars compacta was evaluated using stereology. The results indicate that there is no loss in dopaminergic cell number with age or genotype. However, when mice are challenged with neurotoxic insult by the pesticides paraquat (1, 1'-dimethyl-4,4'-bipyridinium) and maneb (manganese ethylenebisdithiocarbamate), two agents that are effective in inducing selective and irreversible loss of tyrosine hydroxylase-positive neurons in the substantia nigra that is akin to what is seen in Parkinson's disease, there is significant cell loss in the 12 month old H-ferritin-deficient mice compared to control. A surprising finding was that H-ferritin was not detectable in the substantia nigra pars compacta at 4, 8, or 12 months of age regardless of genotype or toxin exposure. The lack of detectable H-ferritin in the substantia nigra opened the possibility that another ferritin, such as mitochondrial ferritin (FtMt), could be an anti-oxidant protein in the brain. FtMt has ferroxidase activity and is similar to H-ferritin except for its cellular localization to the mitochondria. Mitochondrial dysfunction has been linked to Parkinson's disease, which further makes mitochondrial ferritin a protein of interest. We found FtMt expression in almost all regions of the brain, although staining intensity varies between regions. Both neurons and oligodendrocytes prominently immunostain for mitochondrial ferritin. Mice deficient in H-ferritin do not differ in the mitochondrial ferritin staining pattern or intensity as compared to C57/BL6 mice, which further supports existing data suggesting that these two proteins are distinct. Given the importance and relationship between iron and mitochondrial activity, understanding the role of mitochondrial ferritin in neurologic disorders may be important. We explored the expression of FtMt in Restless legs syndrome (RLS), a disorder that involves decreased iron availability in the brain. FtMt levels in human autopsy samples from the putamen and substantia nigra revealed that the RLS nigra had significantly more FtMt than controls; there was not a significant difference in FtMt in the putamen. Immunohistochemical analysis indicated that neuromelanin-containing neurons were the predominant cell type expressing FtMt, and staining intensity in the neurons from the RLS samples was consistently greater than that seen in controls. In addition, cytochrome c oxidase staining, reflective of the number of mitochondria, followed a similar staining pattern to FtMt. The increased number of mitochondria in neurons in RLS coupled with increased FtMt could contribute to insufficient cytosolic iron levels in RLS neurons and the symptoms of this disorder. Overall, the data presented in this thesis suggest that alterations in ferritin levels, despite relatively low levels of expression of both H-ferritin and mitochondrial ferritin, have a wide-ranging impact on cellular energetics and metabolism. The H-ferritindeficient mice are a model in which the study of iron mismanagement can be used to study neurological disorders such as Parkinson's disease and may be a model for RLS.