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Molecular and cellular aspects of protein misfolding and disease
Author(s) -
Herczenik Eszter,
Gebbink Martijn F. B. G.
Publication year - 2008
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fj.07-099671
Subject(s) - protein folding , computational biology , function (biology) , protein aggregation , biology , sequence (biology) , microbiology and biotechnology , chemistry , biochemistry
ABSTRACT Proteins are essential elements for life. They are building blocks of all organisms and the operators of cellular functions. Humans produce a repertoire of at least 30,000 different proteins, each with a different role. Each protein has its own unique sequence and shape (native conformation) to fulfill its specific function. The appearance of incorrectly shaped (misfolded) proteins occurs on exposure to environmental changes. Protein misfolding and the subsequent aggregation is associated with various, often highly debilitating, diseases for which no sufficient cure is available yet. In the first part of this review we summarize the structural composition of proteins and the current knowledge of underlying forces that lead proteins to lose their native structure. In the second and third parts we describe the molecular and cellular mechanisms that are associated with protein misfolding in disease. Finally, in the last part we portray recent efforts to develop treatments for protein misfolding diseases.—Herczenik, E., and Gebbink, M. F. B. G. Molecular and cellular aspects of protein misfolding and disease. FASEB J. 22, 2115–2133 (2008)