Lysosomal glycosphingolipid catabolism by acid ceramidase: Formation of glycosphingoid bases during deficiency of glycosidases

FEBS Letters

Volumn 590, Issue 6, 2016, Pages 716-725

  Ferraz, Maria J ^a   Marques, André R A ^a   Appelman, Monique D ^a   Verhoek, Marri ^b   Strijland, Anneke ^a   Mirzaian, Mina ^b   Scheij, Saskia ^a   Ouairy, Cécile M ^b   Lahav, Daniel ^b   Wisse, Patrick ^b   Overkleeft, Herman S ^b   Boot, Rolf G ^b   Aerts, Johannes M ^a,b  

^a ACADEMIC MEDICAL CENTER   (Netherlands)

^b LEIDEN UNIVERSITY   (Netherlands)

Author keywords

acid ceramidase; Fabry disease; Gaucher disease; globotriaosylsphingosine; glucosylsphingosine; glycosphingolipids

Indexed keywords

ACID CERAMIDASE; GLOBOTRIAOSYLSPHINGOSINE; GLUCOSYLSPHINGOSINE; GLYCOSPHINGOLIPID; LYSOSOMAL GLYCOSPHINGOLIPID; SPHINGOSINE; UNCLASSIFIED DRUG; ALPHA GALACTOSIDASE; ALPHA-GALACTOSIDASE A, HUMAN; ASAH1 PROTEIN, HUMAN; GLUCOSYLCERAMIDASE;

ALLELE; ARTICLE; CELL CULTURE; CONTROLLED STUDY; DEACYLATION; EMBRYO; ENZYME INHIBITION; FABRY DISEASE; FEMALE; FIBROBLAST; GAUCHER DISEASE; GENE LOSS; GENETIC RECOMBINATION; GLA GENE; GLYCOSPHINGOLIPID CATABOLISM; HOMOZYGOSITY; HUMAN; HUMAN CELL; LIPID BLOOD LEVEL; LIPOLYSIS; MALE; MOUSE; NONHUMAN; PATHOGENESIS; PHENOTYPE; PRIORITY JOURNAL; ACYLATION; ANIMAL; CHEMISTRY; DISEASE MODEL; GENETICS; HEK293 CELL LINE; KNOCKOUT MOUSE; LYSOSOME; METABOLISM;

ACID CERAMIDASE; ACYLATION; ALPHA-GALACTOSIDASE; ANIMALS; DISEASE MODELS, ANIMAL; FABRY DISEASE; FEMALE; GAUCHER DISEASE; GLUCOSYLCERAMIDASE; GLYCOSPHINGOLIPIDS; HEK293 CELLS; HUMANS; LYSOSOMES; MALE; MICE; MICE, KNOCKOUT;

EID: 84962286842     PISSN: 00145793     EISSN: 18733468     Source Type: Journal    
DOI: 10.1002/1873-3468.12104     Document Type: Article

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