PROTEIN MISFOLDING AND DISEASE

By DANIEL PEREZ

BASIC INTRODUCTION TO IN VIVO PROTEIN FOLDING AND ITS DEFECTS CYSTIC FIBROSIS 1-ANTITRYPSIN DEFICIENCY PARKINSON S DISEASE ABERRANT PROTEIN FOLDING AS THE MOLECULAR BASIS OF CANCER YEAST AS A MODEL SYSTEM TO INVESTIGATE PROTEIN CONFORMATIONAL DISEASES EXPRESSION OF RECOMBINANT PROTEINS EXPRESSION IN E COLI SYSTEMS PROTEIN EXPRESSION IN YEASTS SITE-DIRECTED MUTAGENESIS PULSE-CHASE LABELING TECHNIQUES FOR THE ANALYSIS OF PROTEIN MATURATION AND DEGRADATION DETECTION OF AGGREGATES AND PROTEIN INCLUSIONS BY STAINING OF TISSUES STUDY OF MUTANT PROTEINS WITH FOLDING DEFECTS IN CULTURED PATIENT CELLS E COLI EXPRESSION SYSTEM FOR IDENTIFYING FOLDING MUTATIONS OF HUMAN ADENOSINE DEAMINASE CHARACTERIZATION OF OVEREXPRESSED MUTANT PROTEINS IN MAMMALIAN CELLS INVESTIGATION OF UNFOLDED-PROTEIN RESPONSE IN CELLS EXPRESSING FAMILIAL ALZHEIMER S DISEASE-LINKED PRESENILIN VARIANTS HUNTINGTIN FRAGMENTS FORM AGGRESOME-LIKE INCLUSION BODIES IN MAMMALIAN CELLS APPLICATION OF CHEMICAL CHAPERONES TO THE RESCUE OF FOLDING DEFECTS ANALYSIS OF DEFECTIVE SUBUNIT INTERACTIONS USING THE TWO-HYBRID SYSTEM INVESTIGATION OF FOLDING AND DEGRADATION OF IN VITRO SYNTHESIZED MUTANT PROTEINS IN THE CYTOSOL INVESTIGATION OF FOLDING AND DEGRADATION OF IN VITRO SYNTHESIZED MUTANT PROTEINS IN MICROSOMES INVESTIGATION OF FOLDING AND DEGRADATION OF IN VITRO SYNTHESIZED MUTANT PROTEINS IN MITOCHONDRIA INVESTIGATION OF FOLDING AND DEGRADATION OF MUTANT PROTEINS SYNTHESIZED IN SEMIPERMEABILIZED CELLS