title: Exocytosis of storage material in a lysosomal disorder.
abstract: Lysosomal exocytosis is a ubiquitously occurring process, which has a physiological role in repair of wounds of the plasma membrane. Lysosomal storage disorders are a group of more than 40 different diseases, which are characterized by intralysosomal storage of various substances. Metachromatic leukodystrophy is a lysosomal disease caused by the deficiency of arylsulfatase A, which results in the storage of the sphingolipid 3-O-sulfogalactosylceramide (sulfatide) in, e.g., oligodendrocytes and distal tubule kidney cells. Here we show that sulfatide storing cultured primary kidney cells of arylsulfatase A deficient mice can undergo calcium induced lysosomal exocytosis and that this results in the delivery of storage material to the culture medium. In metachromatic leukodystrophy extracellular sulfatide has been found in urine and cerebrospinal fluid. Lysosomal exocytosis may explain the presence of sulfatide in these body fluids.
authors.affiliation: Institut für Physiologische Chemie, Rheinische Friedrich-Wilhelms-Universität, Bonn, Germany.
authors.forename: Diana
authors.initials: D
authors.lastname: Klein
authors.forename: Heinrich
authors.initials: H
authors.lastname: Büssow
authors.forename: Simon Ngamli
authors.initials: SN
authors.lastname: Fewou
authors.forename: Volkmar
authors.initials: V
authors.lastname: Gieselmann
mesh_terms: D000818:Animals; D001192:Arylsulfatases / Q000172:deficiency / Q000378:metabolism / Q000494:pharmacology; D002118:Calcium / Q000378:metabolism; D002478:Cells, Cultured; D005089:Exocytosis / Q000502:physiology*; D005699:Galactosylceramides / Q000737:chemistry / Q000378:metabolism; D007686:Kidney Tubules, Distal / Q000378:metabolism; D007966:Leukodystrophy, Metachromatic / Q000378:metabolism; D016464:Lysosomal Storage Diseases / Q000378:metabolism*; D008247:Lysosomes / Q000378:metabolism*; D051379:Mice; D018345:Mice, Knockout; D008817:Mice, Mutant Strains; D009836:Oligodendroglia / Q000378:metabolism; D013433:Sulfoglycosphingolipids / Q000378:metabolism
chemical_list: D005699:Galactosylceramides; D013433:Sulfoglycosphingolipids; D001192:Arylsulfatases; D002118:Calcium