Ryanodine receptor antagonists adapt NPC1 proteostasis to ameliorate lipid storage in Niemann-Pick type C disease fibroblasts

Human Molecular Genetics

Volumn 21, Issue 14, 2012, Pages 3205-3214

  Yu, Ting ^a   Chung, Chan ^a   Shen, Dongbiao ^a   Xu, Haoxing ^a   Lieberman, Andrew P ^a  

^a UNIVERSITY OF MICHIGAN MEDICAL SCHOOL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

1,1' DIHEPTYL 4,4' BIPYRIDIUM; CALCIUM CHANNEL BLOCKING AGENT; CALNEXIN; CELL PROTEIN; CHOLESTEROL; NPC1 PROTEIN; RYANODINE RECEPTOR; SPHINGOLIPID; UNCLASSIFIED DRUG;

ALLELE; ARTICLE; CALCIUM CELL LEVEL; CELL MIGRATION; CONTROLLED STUDY; ENDOPLASMIC RETICULUM; ENDOSOME; FIBROBLAST; HUMAN; HUMAN CELL; LIPID STORAGE; LYSOSOME; MISSENSE MUTATION; NIEMANN PICK DISEASE; NULL ALLELE; PRIORITY JOURNAL; PROTEIN FOLDING; PROTEIN HOMEOSTASIS; STEADY STATE;

CALCIUM CHANNEL BLOCKERS; CARRIER PROTEINS; CELLS, CULTURED; ENDOSOMES; FIBROBLASTS; HUMANS; LIPID METABOLISM; LYSOSOMES; MEMBRANE GLYCOPROTEINS; MUTATION, MISSENSE; NIEMANN-PICK DISEASE, TYPE C; PROTEIN TRANSPORT; PROTEOLYSIS; PROTEOME; RYANODINE RECEPTOR CALCIUM RELEASE CHANNEL;

EID: 84864020267     PISSN: 09646906     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/dds145     Document Type: Article

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