Preface

**Section 3**

**Section 4**

*by Robin Warner*

in Health and Disease

**Section 5**

**Section 6**

**II**

Polyglutamine Pathology, Huntington's Disease and Stem

Molecular Mechanisms of Polyglutamine Pathology and

*Nicole Caroline Mambelli-Lisboa and Eduardo Osorio Frare*

Electrophysiological Investigations of Prion Protein Roles

Molecular Mechanisms behind Initiation of Focal Seizure

in Temporal Lobe Epilepsy: Computational Study *by Ruchi Jakhmola Mani and Deepshikha Pande Katare*

*by Simote T. Foliaki, Bradley R. Groveman and Cathryn L. Haigh*

Lessons Learned from Huntington's Disease

*by Nagehan Ersoy Tunalı*

Huntington's Disease Therapy

Amyotrophic Lateral Sclerosis

Cell Approach in Therapy **85**

**Chapter 7 87**

**Chapter 8 103**

Strategies **119**

**Chapter 9 121**

Prions in Health and Disease **137**

**Chapter 10 139**

Neurodegenerative Processes in Epilepsy **153**

**Chapter 11 155**

Brain-Derived Neurotrophic Factor and Stem Cell-Based Technologies in

*by Irina Kerkis, Joyce Macedo da Silva, Cristiane Valverde Wenceslau,* 

Amyotrophic Lateral Sclerosis: Its Genetics and Targeted Treatment

Neurodegeneration is a broad term used to describe a range of processes that result in dysfunction and eventually death of the neurons. Neurodegenerative diseases represent a very large group of heterogeneous disorders affecting specific subtypes of neurons in the brain. Neurodegenerative diseases are listed among the leading causes of death and pose a serious public health challenge. Recently, the rising global burden of incurable neurodegenerative diseases has forced governments to allocate their financial and scientific resources to neurodegeneration research all around the world.

This book contributes both to the awareness of the anatomy and functions of our mysterious brain and its neurodegenerative states. In the first section of the book, our mini-brain, the cerebellum and its involvement in neurodegeneration are introduced first, since it has major roles in coordination, posture, balance, and important mental processes. In addition to that, the brain stress system and its contribution to neurodegeneration are discussed in a separate chapter. This section is finalized with a chapter involving biomarkers in emergency medicine and neurodegeneration, which will help clinicians and researchers search for certain emergency biomarkers in neurodegenerative conditions. The proceeding sections of the book include the presentation of three widely known and extensively studied neurodegenerative diseases; Alzheimer's Disease, Huntington's Disease, and Amyotrophic Lateral Sclerosis. The main molecular mechanisms, related biomarkers, and current modern therapy strategies are introduced. In addition to those, the characteristics of prion proteins in healthy cellular states and their involvement in prion diseases are discussed. In the last section of the book, molecular mechanisms of epilepsy are introduced, based on the fact that it is commonly associated with various neurodegenerative alterations in brain areas involved in electrographic seizures.

The book is intended to serve as a source to aid clinicians and researchers in the field, and also life science readers to increase their understanding and awareness of the clinical correlations, genetic aspects, neuropathological findings, and current therapeutic interventions in neurodegenerative diseases.

I would like to thank all the authors for their close cooperation and valuable work. I believe that this book will enlighten the curiosity for neurodegeneration and also encourage researchers to work on potentially effective molecular therapies for still mysterious neurodegenerative disorders.

> **Nagehan Ersoy Tunali, Ph.D.** Faculty of Engineering and Natural Sciences, Department of Molecular Biology and Genetics, İstanbul Medeniyet University, İstanbul, Turkey

**1**

Section 1

Brain and Its

Neurodegeneration

Section 1
