This book provides a cutting-edge review of polyglutamine disorders. It primarily focuses on two main aspects: (1) the mechanisms underlying the pathologies' development and progression, and (2) the therapeutic strategies that are currently being explored to stop or delay disease progression. Polyglutamine (polyQ) disorders are a group of inherited neurodegenerative diseases with a fatal outcome that are caused by an abnormal expansion of a coding trinucleotide repeat (CAG), which is then translated in an abnormal protein with an elongated glutamine tract (Q). To date, nine polyQ disorders have been identified and described: dentatorubral-pallidoluysian atrophy (DRPLA); Huntington's disease (HD); spinal-bulbar muscular atrophy (SBMA); and six spinocerebellar ataxias (SCA 1, 2, 3, 6, 7, and 17). The genetic basis of polyQ disorders is well established and described, and despite important advances that have opened up the possibility of generating genetic models of the disease, the mechanisms that cause neuronal degeneration are still largely unknown and there is currently no treatment available for these disorders. Further, it is believed that the different polyQ may share some mechanisms and pathways contributing to neurodegeneration and disease progression.

This book presents key recent developments in biofuel policy, products, processes, patents and innovative technologies. It presents several case studies, which maximize reader insights into how innovative green energy technologies can be implemented on an industrial scale, with illustrations, photos and new approaches. It also analyzes in detail several different technological aspects of the research into and production of green fuels from the first, second and third generation, such as, bioethanol, biogas, biohydrogen, biobutanol, biofuels from pyrolysis, and discusses their economic and environmental impacts. A new source of information for engineers, technicians and students involved in production and research in the biofuels sector, this book also provides a valuable resource for industry, covering the current and future status of biofuels.

This book discusses in what sense constitutional law has a political dimension, raising the question whether constitutional law is fundamentally political as to its validity, terms of its origin, conceptual structure and/or corresponding practice. It also poses the question whether that dimension is a political-theological dimension. A positive answer to these questions challenges the prevailing view that constitutional law is to be conceived strictly as law, moreover as written law, approved at a certain point in history by a particular power and interpreted as any other law by the judiciary. The essays included in this book, written by leading scholars in constitutional theory - including Martin Loughlin, Paul Kahn, Manon Altwegg-Boussac and Massimo La Torre - address these questions in a timely and original way.

Metal Oxide Nanostructures: Synthesis, Properties and Applications covers the theoretical and experimental aspects related to design, synthesis, fabrication, processing, structural, morphological, optical and electronic properties on the topic. In addition, it reviews surface functionalization and hybrid materials, focusing on the advantages of these oxide nanostructures. The book concludes with the current and future prospective applications of these materials. Users will find a complete overview of all the important topics related to oxide nanostructures, from the physics of the materials, to its application.

This volume offers different perspectives on judicial practice in the European and American contexts, both arguably characterized in the last decades by the emergence of novel normative and even policy arguments by judges. The central question deserving the attention of the contributors concerns the degree in which judicial exercises in practical reasoning may amount to forms of judicial usurpation of the legislative function by courts. Since different views as to the nature and scope of legal reasoning lead to different degrees of tolerance regarding what should be admissible to courts, that same nature and scope is thoroughly debated. The main disciplinary approach is that of general jurisprudence, but the contributions take stock of other disciplines in which judicial activism has been addressed, namely positive theories of judicial behavior. Accordingly, the book also explores the development of interdisciplinary dialogue about the theme.

This volume considers how Black activism in Latin America has taken place in varying arenas such as in the academy, digital platforms, and traditional forms of activism. Contributors also examine the impact of activism on policy advocacy and legislation, as well as groups who the Black Lives Matter movement focus on such as women and immigrants. The first part of the book focuses on making Black Lives Matter in academic studies, governmental data, and politics. The next section focuses on the impact of Black activism on policy and legislation in Brazil, Colombia, and Peru. Black activists have been fighting for Black lives throughout Latin America and their struggles have not been in vain, although less policy change has occurred in Peru. The last section finds that social media has allowed for more independent forms of Black activism in Brazil and Cuba.

This book discusses in what sense constitutional law has a political dimension, raising the question whether constitutional law is fundamentally political as to its validity, terms of its origin, conceptual structure and/or corresponding practice. It also poses the question whether that dimension is a political-theological dimension. A positive answer to these questions challenges the prevailing view that constitutional law is to be conceived strictly as law, moreover as written law, approved at a certain point in history by a particular power and interpreted as any other law by the judiciary. The essays included in this book, written by leading scholars in constitutional theory - including Martin Loughlin, Paul Kahn, Manon Altwegg-Boussac and Massimo La Torre - address these questions in a timely and original way.

This book provides a cutting-edge review of polyglutamine disorders. It primarily focuses on two main aspects: (1) the mechanisms underlying the pathologies' development and progression, and (2) the therapeutic strategies that are currently being explored to stop or delay disease progression. Polyglutamine (polyQ) disorders are a group of inherited neurodegenerative diseases with a fatal outcome that are caused by an abnormal expansion of a coding trinucleotide repeat (CAG), which is then translated in an abnormal protein with an elongated glutamine tract (Q). To date, nine polyQ disorders have been identified and described: dentatorubral-pallidoluysian atrophy (DRPLA); Huntington's disease (HD); spinal-bulbar muscular atrophy (SBMA); and six spinocerebellar ataxias (SCA 1, 2, 3, 6, 7, and 17). The genetic basis of polyQ disorders is well established and described, and despite important advances that have opened up the possibility of generating genetic models of the disease, the mechanisms that cause neuronal degeneration are still largely unknown and there is currently no treatment available for these disorders. Further, it is believed that the different polyQ may share some mechanisms and pathways contributing to neurodegeneration and disease progression.

This book presents key recent developments in biofuel policy, products, processes, patents and innovative technologies. It presents several case studies, which maximize reader insights into how innovative green energy technologies can be implemented on an industrial scale, with illustrations, photos and new approaches. It also analyzes in detail several different technological aspects of the research into and production of green fuels from the first, second and third generation, such as, bioethanol, biogas, biohydrogen, biobutanol, biofuels from pyrolysis, and discusses their economic and environmental impacts. A new source of information for engineers, technicians and students involved in production and research in the biofuels sector, this book also provides a valuable resource for industry, covering the current and future status of biofuels.

Perturbation of the boundary is a rather neglected topic in the study of PDEs for two main reasons. First, on the surface it appears trivial, merely a change of variables and an application of the chain rule. Second, carrying out such a change of variables frequently results in long and difficult calculations. In this book, first published in 2005, the author carefully discusses a calculus that allows the computational morass to be bypassed, and he goes on to develop more general forms of standard theorems, which help answer a wide range of problems involving boundary perturbations. Many examples are presented to demonstrate the usefulness of the author's approach, while on the other hand many tantalizing open questions remain. Anyone whose research involves PDEs will find something of interest in this book.