Genetic errors may result in abnormal synthesis of proteins,abnormal folding and processing of proteins after they are synthesized,or changes in the functional properties of proteins. Althoughthe synthesis of proteins begins in the cytoplasm, many aredestined to be expressed on the cell surface (e.g., surface-recognitionmolecules, ion channels, receptors, and adhesion molecules)or secreted (e.g., hormones, growth factors, extracellular matrixproteins, and proteolytic enzymes). Newly synthesized membraneand secretory proteins are transported into the endoplasmicreticulum, the internal membranous network of the cell, in alargely unfolded state. When they leave the endoplasmic reticulumto move through . . . [Full Text of this Article]
Molecular Chaperones in the Endoplasmic Reticulum
Synthesis of Molecular Chaperones
The Role of Chaperones in the Maturation of Newly Synthesized Proteins
The Fate of Misfolded Proteins
Ischemia as an Example of Stress That Causes Abnormal Protein Folding
Inherited Alterations in Protein Folding and Assembly
Cystic Fibrosis
Alpha1-Antitrypsin Deficiency
Conclusions
Source Information
From the Renal Division, Department of Medicine, Brigham and Women's Hospital, and Harvard Medical School, Boston (G.K., S.K.N.); and the Eisai Research Institute, Andover, Mass. (G.K.).
Address reprint requests to Dr. Nigam at the Harvard Institutes of Medicine, 77 Ave. Louis Pasteur, Boston, MA 02115.
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