Question

Problem 5. (15 pts) Prion proteins are involved in several neurological diseases associated with the formation of fibrils and protein aggregates in the brain. (a) What is the proposed mechanism for the change in the protein that causes disease? (b) Draw the free energy diagram corresponding to this process.

Answer 1

Doelle allee Aggregate at caused the Pape are disease. Popse model This change due I the x-helix. to change in x-Hellre is amino and sequenee incorporited into B-sheet.. High energy become randoness (high entropy Intermediated. energy has the low native protein energy. centropy) The aggrigate 1 Verey low of protein structure are the entropy because their surface higher energy.c higher e area are less. Polding intermediat, enrgy intermediate Amophors I aggrigate Mative - structure Amyloid fibre

no are lack nuclic usease aid. The causing agents prion has been seemed traced to to which Prusiner doubbed prion protein (Pop) name are derived Protein aceous infection. Prion protein is a normal constituent of brain. tissue in all mammals. Illness occure only when the normal cellular Pap or Prp. occure in an altered conformation called Porpsc (sc= scrapie) The structure of Pope contain 22-helise. The infection of Prpse with Pope converts the latter to prpc initating domain effect in which more of the brain protein converted to disease causing form. change of the one amino and in Ppp is converte prosc are delivered. Pop is beginning to provide insight maleculant process that allow the interact so as to alter their into the prion protein conformation. s Heli's Helix B Helix-B cewe Eeeee 2 relles 3 Helive leece Melixe Aggrigate. Prpse