Visualization of cholesterol deposits in lysosomes of Niemann-Pick type C fibroblasts using recombinant perfringolysin O

Orphanet Journal of Rare Diseases

Volumn 9, Issue 1, 2014, Pages

  Kwiatkowska, Katarzyna ^a   Marszałek Sadowska, Ewelina ^a   Traczyk, Gabriela ^a   Koprowski, Piotr ^a   Musielak, Małgorzata ^b   Ługowska, Agnieszka ^b   Kulma, Magdalena ^c   Grzelczyk, Anna ^a   Sobota, Andrzej ^a  

^a NENCKI INSTITUTE OF EXPERIMENTAL BIOLOGY   (Poland)

^b INSTITUTE OF PSYCHIATRY AND NEUROLOGY   (Poland)

^c INSTITUTE OF BIOCHEMISTRY AND BIOPHYSICS   (Poland)

Author keywords

Cholesterol binding proteins; Filipin; Lipid storage diseases; Niemann Pick C disease; Perfringolysin O

Indexed keywords

CERAMIDE; CHITOTRIOSIDASE; CHOLESTEROL; FILIPIN; GLUTATHIONE TRANSFERASE; GLUTATHIONE TRANSFERASE PERFRINGOLYSIN O PROTEIN FUSION; LYSOBISPHOSPHATIDIC ACID; LYSOSOME ASSOCIATED MEMBRANE PROTEIN 1; METHYL BETA CYCLODEXTRIN; RECOMBINANT PROTEIN; SPHINGOMYELIN; STREPTAVIDIN; TRITON X 100; UNCLASSIFIED DRUG;

ACTIN FILAMENT; ARTICLE; CELL ORGANELLE; CONTROLLED STUDY; ENDOPLASMIC RETICULUM; ENDOSOME; ENZYME ACTIVITY; ENZYME LINKED IMMUNOSORBENT ASSAY; FIBROBLAST; GOLGI COMPLEX; HUMAN; HUMAN CELL; IMMUNOELECTRON MICROSCOPY; IMMUNOFLUORESCENCE; LYSOSOME; MOLECULAR PROBE; NIEMANN PICK DISEASE; PEROXISOME; PROTEIN LOCALIZATION; SURFACE PLASMON RESONANCE;

BACTERIAL TOXINS; CASE-CONTROL STUDIES; CHOLESTEROL; ENZYME-LINKED IMMUNOSORBENT ASSAY; FIBROBLASTS; FLUORESCENT ANTIBODY TECHNIQUE; HEMOLYSIN PROTEINS; HUMANS; LYSOSOMES; MICROSCOPY, ELECTRON; NIEMANN-PICK DISEASE, TYPE C; RECOMBINANT PROTEINS; SURFACE PLASMON RESONANCE;

EID: 84899976153     PISSN: None     EISSN: 17501172     Source Type: Journal    
DOI: 10.1186/1750-1172-9-64     Document Type: Article

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