Innovative strategies to treat protein misfolding in inborn errors of metabolism: Pharmacological chaperones and proteostasis regulators

Journal of Inherited Metabolic Disease

Volumn 37, Issue 4, 2014, Pages 505-523

  Muntau, Ania C ^a   Leandro, João ^b   Staudigl, Michael ^a   Mayer, Felix ^a   Gersting, Søren W ^a  

^a LUDWIG MAXIMILIANS UNIVERSITY   (Germany)

^b RESEARCH INSTITUTE FOR MEDICINES IMED ULISBOA   (Portugal)

Author keywords

[No Author keywords available]

Indexed keywords

ADENOSINE TRIPHOSPHATE; AGENTS AFFECTING PROTEIN METABOLISM; CHAPERONE; CHAPERONIN; CHAPERONIN CONTAINING TCP1; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; HEAT SHOCK PROTEIN; HEAT SHOCK PROTEIN 40; HEAT SHOCK PROTEIN 70; HEAT SHOCK PROTEIN 90; SAPROPTERIN; TAFAMIDIS; PROTEIN;

CALCIUM SIGNALING; CONFERENCE PAPER; CYSTIC FIBROSIS; ENVIRONMENTAL STRESS; ENZYME REPLACEMENT; FAMILIAL AMYLOIDOSIS; GAUCHER DISEASE; HEAT SHOCK RESPONSE; HUMAN; INBORN ERROR OF METABOLISM; LYSOSOME STORAGE DISEASE; METABOLIC DISORDER; METHYLMALONIC ACIDURIA; MISSENSE MUTATION; NONHUMAN; OXALOSIS 1; PHENYLKETONURIA; PROTEIN AGGREGATION; PROTEIN CONFORMATION; PROTEIN DEGRADATION; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN FUNCTION; PROTEIN HOMEOSTASIS; PROTEIN METABOLISM; PROTEIN MISFOLDING; PROTEIN QUALITY; PROTEIN STABILITY; PROTEIN SYNTHESIS; PROTEIN TRANSPORT; THERMOSTABILITY; ANIMAL; CHEMISTRY; COMPLICATION; DRUG DELIVERY SYSTEM; DRUG EFFECTS; ENDOPLASMIC RETICULUM ASSOCIATED DEGRADATION; KINETICS; METABOLISM; METABOLISM, INBORN ERRORS; PHYSIOLOGY; PROTEOSTASIS DEFICIENCIES;

ANIMALS; DRUG DELIVERY SYSTEMS; ENDOPLASMIC RETICULUM-ASSOCIATED DEGRADATION; HUMANS; KINETICS; METABOLISM, INBORN ERRORS; MOLECULAR CHAPERONES; PROTEIN FOLDING; PROTEINS; PROTEOSTASIS DEFICIENCIES;

EID: 84904099631     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-014-9701-z     Document Type: Conference Paper

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