Prion diseases is a neurodegenerative disorder caused by abnormally folded Prion protein in the brain.

Molecular self-assembly of incorrectly folded proteins into insoluble amyloid fibers is responsible for infectious prion-related neurodegenerative diseases.

Acid-Base Homeostasis outside the acceptable range of pH, proteins are denatured (3D structure is disrupted), causing enzymes and ion channels (among others) to malfunction.

http://europepmc.org/article/PMC/4260053