TRA CỨU
thông tin biểu ghi
ISBN 9789813222953
DDC 616.831
Nhan đề Protein folding disorders of the central nervous system / Jorge Ghiso, Agueda Rostagno (editor)
Thông tin xuất bản Singapore : World Scientific, 2018
Mô tả vật lý 334 pages. : illustrations (some color) ; 24 cm.
Tóm tắt This exciting new book explores the dark side of the molecular protein assembly bringing an updated view of how failures in the homeostatic mechanisms that efficiently regulate protein folding leads to the accumulation of structurally abnormal pathogenic assemblies, encompassing an emerging group of diseases collectively known as "Protein Folding Disorders." This complex and diverse group of chronic and progressive entities are bridged together by their relationship to structural transitions in the native state of specific proteinaceous components, which for reasons poorly understood, convert into polymeric aggregates that generate poorly soluble tissue deposits and which are considered today the culprit of the disease pathogenesis in their respective diseases. Despite the diversity in the amino acid sequence of the different proteins involved in these heterogeneous disorders, all the pathologic conformers can trigger cascades of events ultimately resulting in cell dysfunction and death with devastating clinical consequences in many of the most precious aspects of human existence including personality, cognition, memory, and skilled movements. This book, which is composed of a compilation of chapters authored by outstanding and well-published scientists in the respective fields currently performing active investigations at world renowned universities and research centers, focuses on the growing number of diseases associated with protein misfolding in the central nervous system. Individual chapters are dedicated to the most common neurodegenerative diseases associated with protein aggregation/fibrillization focusing on the nature of the pathogenic species and the cellular pathways involved in the molecular pathogenesis of Alzheimer's, Parkinson's, and Huntington's diseases as well as in Amyotrophic Lateral Sclerosis, and Prion disorders. A group of contributions is centered on the current knowledge of the intracellular pathways and subcellular organelles affected by the different disease conditions, while others are focused in the emerging pathogenic role of misfolded subunits assembled into neurotoxic soluble oligomers, and in the novel notion of the transmissibility of the protein misfolded species, an innovative concept until recently only accepted for Prion diseases. Lastly, a different set of chapters is dedicated to the evaluation of novel therapeutic strategies for these devastating diseases.
Từ khóa tự do Central nervous system
Từ khóa tự do Degenerative neuropathy
Từ khóa tự do Molecular biology of neuropathy
Từ khóa tự do Prion disease
Từ khóa tự do Protein misfolding
Từ khóa tự do Protein disorders
Khoa Khoa Y
Tác giả(bs) CN Ghiso, Jorge
Tác giả(bs) CN Rostagno, Agueda
Địa chỉ 300Q12_Kho Mượn_02(2): 100381-2
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245 |aProtein folding disorders of the central nervous system / |cJorge Ghiso, Agueda Rostagno (editor)
260 |aSingapore : |bWorld Scientific, |c2018
300 |a334 pages. : |billustrations (some color) ; |c24 cm.
520 |aThis exciting new book explores the dark side of the molecular protein assembly bringing an updated view of how failures in the homeostatic mechanisms that efficiently regulate protein folding leads to the accumulation of structurally abnormal pathogenic assemblies, encompassing an emerging group of diseases collectively known as "Protein Folding Disorders." This complex and diverse group of chronic and progressive entities are bridged together by their relationship to structural transitions in the native state of specific proteinaceous components, which for reasons poorly understood, convert into polymeric aggregates that generate poorly soluble tissue deposits and which are considered today the culprit of the disease pathogenesis in their respective diseases. Despite the diversity in the amino acid sequence of the different proteins involved in these heterogeneous disorders, all the pathologic conformers can trigger cascades of events ultimately resulting in cell dysfunction and death with devastating clinical consequences in many of the most precious aspects of human existence including personality, cognition, memory, and skilled movements. This book, which is composed of a compilation of chapters authored by outstanding and well-published scientists in the respective fields currently performing active investigations at world renowned universities and research centers, focuses on the growing number of diseases associated with protein misfolding in the central nervous system. Individual chapters are dedicated to the most common neurodegenerative diseases associated with protein aggregation/fibrillization focusing on the nature of the pathogenic species and the cellular pathways involved in the molecular pathogenesis of Alzheimer's, Parkinson's, and Huntington's diseases as well as in Amyotrophic Lateral Sclerosis, and Prion disorders. A group of contributions is centered on the current knowledge of the intracellular pathways and subcellular organelles affected by the different disease conditions, while others are focused in the emerging pathogenic role of misfolded subunits assembled into neurotoxic soluble oligomers, and in the novel notion of the transmissibility of the protein misfolded species, an innovative concept until recently only accepted for Prion diseases. Lastly, a different set of chapters is dedicated to the evaluation of novel therapeutic strategies for these devastating diseases.
541 |aTặng
653 |aCentral nervous system
653 |aDegenerative neuropathy
653 |aMolecular biology of neuropathy
653 |aPrion disease
653 |aProtein misfolding
653|aProtein disorders
690 |aKhoa Y
700 |aGhiso, Jorge|eeditor
700 |aRostagno, Agueda|eeditor
852|a300|bQ12_Kho Mượn_02|j(2): 100381-2
8561|uhttp://elib.ntt.edu.vn/documentdata01/2 tailieuthamkhao/600 congnghe/biasach_2025_1/57544_proteinthumbimage.jpg
890|a2|b0|c0|d0
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