Accumulation of sialic acid in endocytic compartments interferes with the formation of mature lysosomes. Impaired proteolytic processing of cathepsin B in fibroblasts of patients with lysosomal sialic acid storage disease

Journal of Biological Chemistry

Volumn 274, Issue 27, 1999, Pages 19063-19071

  Schmid, Johannes A ^a,b   Mach, Lukas ^a,c   Paschke, Eduard ^d   Glössl, Josef ^a  

^a UNIVERSITY OF NATURAL RESOURCES AND LIFE SCIENCES   (Austria)

^b UNIVERSITY OF VIENNA   (Austria)

^c UNIVERSITY OF WESTERN AUSTRALIA   (Australia)

^d MEDICAL UNIVERSITY OF GRAZ   (Austria)

Author keywords

[No Author keywords available]

Indexed keywords

CATHEPSIN B; CYSTEINE PROTEINASE; HYDROLASE; LYSOSOME ENZYME; MONOSACCHARIDE; SIALIC ACID; SUCROSE;

ACIDIFICATION; ARTICLE; BIOGENESIS; CELLULAR DISTRIBUTION; CONTROLLED STUDY; DENSITY; ENDOCYTOSIS; ENDOSOME; ENZYME SYNTHESIS; FIBROBLAST; HUMAN; HUMAN CELL; LYSOSOME; LYSOSOME STORAGE DISEASE; PRIORITY JOURNAL;

CATHEPSIN B; CELL COMPARTMENTATION; CELL LINE; ELECTROPHORESIS, POLYACRYLAMIDE GEL; ENDOCYTOSIS; FIBROBLASTS; HUMANS; HYDROGEN-ION CONCENTRATION; LYSOSOMAL STORAGE DISEASES; LYSOSOMES; MOLECULAR WEIGHT; N-ACETYLNEURAMINIC ACID; PHENOTYPE; SUCROSE;

EID: 0033516559     PISSN: 00219258     EISSN: None     Source Type: Journal    
DOI: 10.1074/jbc.274.27.19063     Document Type: Article

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