PROTEIN CONFORMATION AND DISEASE

In a number of neurodegenerative diseases, a change in the conformatio n of specific proteins occurs which is considered to be related to the development of the disease process. In general, a change in the prote in conformation from a predominantly alpha-helical to a predominantly beta-sheet structure, due to genetic or environmental factors, leads t o oligomerization of the proteins. In Alzheimer's and prion diseases, insoluble precipitates or plaques are often observed in vivo as a cons equence of beta-fibril formation due to the polymerization of the prot eins. In certain other neurodegenerative diseases, for example in Hunt ington disease, the beta-sheet oligomers may interact differently with other functional proteins compared to the monomeric proteins inducing the disease. Biophysical studies would provide information regarding the forces responsible for the oligomerization of these proteins and h ence provide methods for detecting these diseases, leading eventually to therapeutic approaches.