Protein Homeostasis Diseases

Mechanisms and Novel Therapies
Herausgeber: Pey, Angel L

• Broschiertes Buch

Produktbeschreibung

Protein Homeostasis Diseases: Mechanisms and Novel Therapies offers an interdisciplinary examination of the fundamental aspects, biochemistry and molecular biology of protein homeostasis disease, including the use of natural and pharmacological small molecules to treat common and rare protein homeostasis disorders. Contributions from international experts discuss the biochemical and genetic components of protein homeostasis disorders, the mechanisms by which genetic variants may cause loss-of-function and gain-of-toxic-function, and how natural ligands can restore protein function and homeostasis in genetic diseases. Applied chapters provide guidance on employing high throughput sequencing and screening methodologies to develop pharmacological chaperones and repurpose approved drugs to treat protein homeostasis disorders.

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Produktdetails

• Verlag: Elsevier Science
• Seitenzahl: 450
• Erscheinungstermin: 19. Februar 2020
• Englisch
• Abmessung: 231mm x 191mm x 25mm
• Gewicht: 885g
• ISBN-13: 9780128191323
• ISBN-10: 0128191325
• Artikelnr.: 58261276

Inhaltsangabe

I. Introduction of protein folding and homeostasis 1. Protein folding: how,
why, and beyond 2. Protein homeostasis and disease
II. Protein folding and homeostasis at the organismal and proteomic scales
3. Caenorhabditis elegans as a model organism for protein homeostasis
diseases 4. Proteome-scale studies of protein stability 5. Classifying
disease-associated variants using measures of protein activity and
stability
III. Protein homeostasis disturbance in disease: Genetics, mechanisms, and
modulation by natural ligands 6. Protein destabilization and degradation as
a mechanism for hereditary disease 7. Detection of amyloid aggregation in
living systems 8. Molecular mechanisms of amyloid aggregation in human
proteinopathies 9. Metals and amyloid gain-of-toxic mechanisms in
neurodegenerative diseases 10. Vitamin B6-dependent enzymes and disease 11.
Galactosemia: opportunities for novel therapies 12. Protein homeostasis and
regulation of intracellular trafficking of G protein-coupled receptors 13.
Structure-guided discovery of pharmacological chaperones targeting protein
conformational and misfolding diseases 14. Virtual screening in drug
discovery: a precious tool for a still-demanding challenge 15. Differential
scanning fluorimetry in the screening and validation of pharmacological
chaperones for soluble and membrane proteins 16. Cellular high-throughput
screening 17. High-throughput screening for intrinsically disordered
proteins by using biophysical methods 18. Natural and pharmacological
chaperones against accelerated protein degradation: uroporphyrinogen III
synthase and congenital erythropoietic porphyria