Molecular pathways for intracellular cholesterol accumulation: Common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosis

Archives of Biochemistry and Biophysics

Volumn 515, Issue 1-2, 2011, Pages 54-63

  Cianciola, Nicholas L ^a   Carlin, Cathleen R ^a   Kelley, Thomas J ^a  

^a CASE WESTERN RESERVE UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Cholesterol homeostasis; Cystic fibrosis; Molecular motors; Niemann Pick disease Type C; Organelle motility; Rab GTPases

Indexed keywords

2 HYDROXYPROPYL BETA CYCLODEXTRIN; 25 HYDROXYCHOLESTEROL; ABC TRANSPORTER A1; CHOLESTEROL; CHOLESTEROL ACYLTRANSFERASE; CYCLIC AMP DEPENDENT PROTEIN KINASE ANCHORING PROTEIN; GUANINE NUCLEOTIDE DISSOCIATION INHIBITOR; GUANINE NUCLEOTIDE EXCHANGE FACTOR; GUANOSINE TRIPHOSPHATASE ACTIVATING PROTEIN; INDUCIBLE NITRIC OXIDE SYNTHASE; LOW DENSITY LIPOPROTEIN CHOLESTEROL; LOW DENSITY LIPOPROTEIN RECEPTOR; LYSOSOME ENZYME; RAB PROTEIN; RAB7 PROTEIN; SOMATOMEDIN B RECEPTOR; STEROL REGULATORY ELEMENT BINDING PROTEIN; TOLL LIKE RECEPTOR 4; TRANSMEMBRANE CONDUCTANCE REGULATOR;

ARTICLE; AUTOPHAGOSOME; BACTERIAL CLEARANCE; BACTERIAL INFECTION; CALCIUM HOMEOSTASIS; CELL MIGRATION; CELL MOTILITY; CELL VACUOLE; CEREBELLUM DISEASE; CHLORIDE TRANSPORT; CHOLESTEROL SYNTHESIS; CHRONIC INFLAMMATION; CONFORMATIONAL TRANSITION; CYSTIC FIBROSIS; CYTOKINE PRODUCTION; ENDOCYTOSIS; ENDOPLASMIC RETICULUM; ENDOSOME; FAMILIAL HYPERCHOLESTEROLEMIA; HEPATOSPLENOMEGALY; HUMAN; INFECTION; INTRACELLULAR SIGNALING; LIPID METABOLISM; LIPID STORAGE; LOSS OF FUNCTION MUTATION; LYSOSOME; MICROTUBULE ORGANIZING CENTER; MUCOCILIARY CLEARANCE; NERVE DEGENERATION; NIEMANN PICK DISEASE; NONHUMAN; PANCREAS INSUFFICIENCY; PRIORITY JOURNAL; PROTEIN DOMAIN; PROTEIN EXPRESSION; PROTEIN FAMILY; PROTEIN FOLDING; PROTEIN PHOSPHORYLATION; PURKINJE CELL; RESPIRATORY FAILURE; SWEAT TEST; TRANS GOLGI NETWORK;

BIOLOGICAL TRANSPORT; CHOLESTEROL; CYSTIC FIBROSIS; HOMEOSTASIS; HUMANS; NIEMANN-PICK DISEASE, TYPE C; SIGNAL TRANSDUCTION;

EID: 80053950498     PISSN: 00039861     EISSN: 10960384     Source Type: Journal    
DOI: 10.1016/j.abb.2011.08.012     Document Type: Article

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