Synopsis
110 of the world's foremost authorities explore the latest advances in molecular and cellular neurobiology and molecular neurogenetics and their implications for the development of pharmacologic or gene therapy for patients with genetic diseases of the nervous system. The 3rd Edition features a new section on psychiatric diseases, 26 additional new chapters, and an even stronger clinical focus, offering practical guidance on a full range of diseases and the roles that molecular biology and genetics play in their diagnosis and management.the latest advances in molecular research.
- Includes a brand-new section on Psychiatric Diseases, edited by Dr. Eric J. Nestler, that features chapters on Challenges in Psychiatric Genetics • Depression • Bipolar Disorders • Schizophrenia • Obsessive-Compulsive Disorder and Tourette's Syndrome • Molecular and Genetic Basis of Addiction • and Autism.
- Offers two new chapters on Degenerative Diseases and Protein Processing and Prion Diseases, authored by Dr. Stanley B. Prusiner, winner of the 1997 Nobel Prize in Medicine for his research defining the molecular and genetic basis of the spongiform encephalopathies and the expression of the prion gene under both physiologic and pathologic conditions.
- Incorporates new data and insights from the analysis and sequencing of the human genome into three new chapters on The Human Genome Project and Neurological Disease, Gene Therapy, and Ethical Issues in Diagnosis and Therapy.
- Features 21 additional new chapters: Animal Models (mice, worms, flies) • Gene Targeting/Gene Mapping • Genotype/Phenotype Correlations • Mitochondrial Disorders Due to Mutations in the Nuclear Genome • Mitochondria in Neurodegenerative Disorders • Lysosomal Membrane Disorders – LAMP-2 Deficiency • Alzheimer's Disease and Related Dementias • Movement Disorders • Neuronopathies • Limb Girdle Muscular Dystrophies • Congenital Myopathies • Hereditary Inclusion – Body Myopathies • Facioscapulohumeral Muscular Dystrophy • The Phakomatoses: Disorders of Skin and Brain • Disorder of Galactose Metabolism • Disorders of Glucose Transport • Congenital Disorders of Glycosylation • Disorders of Glutathione Metabolism • Friedreich Ataxia and Iron Metabolism • The Influence of Alpha Tocopherol, Caloric Restriction and Genes on Life Span • and A Neurologic Gene Map.
About the Authors
Dr. Nestler is the Nash Family Professor of Neuroscience at the Mount Sinai School of Medicine in New York, where he serves as Chair of the Department of Neuroscience and Director of the Friedman Brain Institute. He received his B.A., Ph.D., and M.D. degrees, and psychiatry residency training, from Yale University. He served on the Yale faculty from 1987-2000, where he was the Elizabeth Mears and House Jameson Professor of Psychiatry and Neurobiology, and Director of the Division of Molecular Psychiatry. He moved to Dallas in 2000 where he served as the Lou and Ellen McGinley Distinguished Professor and Chair of the Department of Psychiatry at The University of Texas Southwestern Medical Center until moving to New York in 2008. Dr. Nestler is a member of the Institute of Medicine and a Fellow of the American Academy of Arts and Sciences. The goal of Dr. Nestler’s research is to better understand the molecular mechanisms of addiction and depression based on work in animal models, and to use this information to develop improved treatments of these disorders.
Roger N. Rosenberg, MD is a graduate of Northwestern University Medical School, With Distinction, and was subsequently trained in Neurology with H. Houston Merritt, MD at the Neurological Institute, Columbia University, New York, was Chief Resident and then was a Post-Doctoral Fellow with Nobel Laureate Marshall Nirenberg at the NIH in the Laboratory of Biochemical Genetics. He is Board Certified by the American Board of Psychiatry and Neurology. He is holder of the Zale Distinguished Chair and Professor of Neurology and Neurotherapeutics at the University of Texas Southwestern Medical Center at Dallas since 1973 and developed the department for 18 years as Chair from 1973-1991.
He described for the first time in 1975 Machado Joseph disease, an autosomal dominant cerebellar degeneration, which produces imbalance and impaired coordination, and showed it was due to a unique expansion of DNA in the causal gene. It is the most common inherited form of impaired coordination in the world and his research has provided a genetic marker to eliminate it in large families in future generations.
He has served as the Founding Director of the UT Southwestern NIH funded Alzheimer’s Disease Center and Principal Investigator of the NIH Center Grant from 1987-2019.
He directs an active laboratory effort in Alzheimer’s Disease. He is developing a DNA Aβ42 trimer vaccine for Alzheimer's disease for which he was awarded a US Patent "Amyloid Beta Gene Vaccines" in January 2009. It has been tested in mouse, transgenic mouse, New Zealand white rabbits and rhesus monkeys. The vaccine produces effective anti-Aβ42 peptide antibody levels and is non-inflammatory in all three species. The vaccine reduces by 40% Aβ42 peptide and by 50% tau and phospho-tau in the brains of 3X AD Tg mice, the two main pathologies of Alzheimer’s disease, with high levels of anti-Aβ42 antibody and with a non-inflammatory immune response. He is preparing now a Phase 1 Clinical trial Grant - First in Human to determine its effectiveness and safety in human subjects.
He has published 297 original scientific articles, chapters, reviews, and editorials.
He served as Editor in Chief from 1997 through 2017 for JAMA Neurology (formerly Archives of Neurology), a major international neurology journal, published by the American Medical Association. During his tenure, he raised the Impact Factor of the journal from 3.0 to 10.2, placing JAMA Neurology as #1 of all US publications in neurology.
He is the founding editor of two of the landmark texts in neuroscience. Rosenberg’s Molecular and Genetic Basis of Neurological and Psychiatric Disease, 5th edition, published in 2015 by Elsevier. The 6th edition will publish in 2020. The Atlas of Clinical Neurology, 4th edition, has just been published.
He is a former President of the American Academy of Neurology, former Vice-President of the American Neurological Association, an Honorary Member of both organizations, and a Fellow of the American Association for the Advancement of Science. He received the first Science Medal in 2009 from the World Federation of Neurology for his contributions to neuro-genetics, for his original clinical and molecular genetics research on Machado-Joseph disease, and the development of the DNA Abeta42 trimer vaccine for Alzheimer’s disease.
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