Molecular Chaperones in Human Disorders

Molecular Chaperones in Human Disorders, Volume 114 in the Advances in Protein Chemistry and Structural Biology series, provides an overview of current developments in mechanisms underlying DNA repair and their involvement in maintaining chromatin repair, the balance between chromosomal repair pathways, tumorigenesis, immune signaling and infection-induced inflammation. Chapters in this new release cover Functional principles and regulation of molecular chaperones, Chaperones and retinal disorders, Protein misfolding and degradation in genetic diseases, Chaperone dysfunction in hereditary myopathic diseases, Diseases caused by functional disorder of molecular chaperones residing in the endoplasmic reticulum, and many other timely topics.

Describes advances in our understanding on DNA repair mechanisms and the involvement of their dysregulation in promoting diseasesProvides an ideal resource for a very wide audience of specialists, researchers and studentsContains timely chapters written by well-renown authorities in their fieldPresents information that is well supported by a number of high quality illustrations, figures and tables

1. Functional principles and regulation of molecular chaperones Johannes Buchner 2. Chaperones and retinal disorders Nikolai O. Artemyev 3. Protein misfolding and degradation in genetic diseases Rasmus Hartmann-Petersen 4. Chaperone dysfunction in hereditary myopathic diseases Andreas Roos 5. Diseases caused by functional disorder of molecular chaperones residing in the endoplasmic reticulum Masafumi Sakono 6. Structural and functional insights on the roles of molecular chaperones in the mistargeting and aggregation phenotypes associated with primary hyperoxaluria type I Angel Luis Pey 7. Inflammatory response and its relation to sphingolipid metabolism proteins: Targeting inflammation with molecular chaperones Elif Ozkirimli 8. When safeguarding goes wrong: impact of oxidative stress on proteins homeostasis in health and neurodegenerative disorders Dana Reichmann 9. Computational approach to unravel the misfolding mechanism of Glucosylceramidase mutations in Gaucher Disease George Priya Doss P. C 10. Cytosolic quality control proteins, SGTA and the Bag6 complex, in disease Rivka L. Isaacson