Modern pathology: protein mis-folding and mis-processing in complex disease.
Electrostatic and electrochemical properties of bio-molecules, such as proteins, are governed by energy parameters that are, in part dependent on its folding. Disruption of this process can lead to the development of complex, multisystem diseases whose presentation may be organ-dependent. Examples include cystic fibrosis, alpha-1 antitrypsin deficiency, and Alzheimer disease. In addition to explaining exotic pathologic syndromes, an understanding of protein folding mechanisms may facilitate the understanding of less complex diseases and allow the development of novel therapeutic approaches.
Fadiel
Ahmed
A
Ob/Gyn Department, New York University School of Medicine, New York, NY 10016, USA. ahmed.fadiel@med.nyu.edu
Eichenbaum
Kenneth D
KD
Hamza
Adel
A
Tan
Orkon
O
Lee
Hae H
HH
Naftolin
Frederick
F
D000544:Alzheimer Disease / Q000378:metabolism; D000595:Amino Acid Sequence; D000818:Animals; D007562:Creutzfeldt-Jakob Syndrome / Q000235:genetics / Q000378:metabolism; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism; D004563:Electrochemistry; D019143:Evolution, Molecular; D006801:Humans; D008958:Models, Molecular; D008969:Molecular Sequence Data; D046912:Multiprotein Complexes; D011328:Prions / Q000737:chemistry / Q000235:genetics; D017510:Protein Folding; D011499:Protein Processing, Post-Translational; D011506:Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D040901:Proteomics; D017386:Sequence Homology, Amino Acid; D055672:Static Electricity; D013577:Syndrome
D046912:Multiprotein Complexes; D011328:Prions; D011506:Proteins