1. These disorders involve failure of protein structural stabilization or degradation by specialized proteins known as chaperones. Important chaperones include heat shock proteins induced by stress, one of which is ubiquitin, which marks abnormal proteins for degradation.
  2.  
    Two known pathogenetic mechanisms include:
    1. Abnormal protein aggregation, which is characteristic of amyloidosis; a number of neurodegenerative diseases, such as Alzheimer disease, Huntington disease, and Parkinson disease; and perhaps prion diseases, such as “mad cow” disease
    2.  
      Abnormal protein transport and secretion, which is characteristic of cystic fibrosis and α1-antitrypsin deficiency