Mannose 6-phosphate receptor-dependent endocytosis of lysosomal enzymes is increased in sulfatide-storing kidney cells.
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Mannose 6-phosphate receptor-dependent endocytosis of lysosomal enzymes is increased in sulfatide-storing kidney cells. / Klein, Diana; Yaghootfam, Afshin; Matzner, Ullrich; Koch, Bettina; Braulke, Thomas; Gieselmann, Volkmar.
In: BIOL CHEM, Vol. 390, No. 1, 1, 2009, p. 41-48.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Mannose 6-phosphate receptor-dependent endocytosis of lysosomal enzymes is increased in sulfatide-storing kidney cells.
AU - Klein, Diana
AU - Yaghootfam, Afshin
AU - Matzner, Ullrich
AU - Koch, Bettina
AU - Braulke, Thomas
AU - Gieselmann, Volkmar
PY - 2009
Y1 - 2009
N2 - Metachromatic leukodystrophy is a lysosomal disorder caused by the deficiency of arylsulfatase A (ASA). This leads to the storage of the sphingolipid 3-O-sulfogalactosylceramide (sulfatide) in various cell types, such as renal tubular cells. Examination of mannose 6-phosphate receptor (MPR300)-dependent endocytosis revealed that uptake of lysosomal enzymes is more than two-fold increased in sulfatide-storing kidney cells. Expression of MPR300 and its internalization rate is increased in these cells, whereas the recycling rate is decreased. Similar alterations can be found for the transferrin receptor, indicating that sulfatide storage leads to a general alteration of the endocytotic pathway. These data allow calculating that the endosomal pool from which receptors can recycle is 1.4- to 2-fold increased in lipid-storing cells. Immunocytochemistry demonstrates that the MPR300 in lipid-storing cells does not co-localize with accumulated sulfatide, suggesting that the kinetics of internalization and recycling appear to be altered indirectly.
AB - Metachromatic leukodystrophy is a lysosomal disorder caused by the deficiency of arylsulfatase A (ASA). This leads to the storage of the sphingolipid 3-O-sulfogalactosylceramide (sulfatide) in various cell types, such as renal tubular cells. Examination of mannose 6-phosphate receptor (MPR300)-dependent endocytosis revealed that uptake of lysosomal enzymes is more than two-fold increased in sulfatide-storing kidney cells. Expression of MPR300 and its internalization rate is increased in these cells, whereas the recycling rate is decreased. Similar alterations can be found for the transferrin receptor, indicating that sulfatide storage leads to a general alteration of the endocytotic pathway. These data allow calculating that the endosomal pool from which receptors can recycle is 1.4- to 2-fold increased in lipid-storing cells. Immunocytochemistry demonstrates that the MPR300 in lipid-storing cells does not co-localize with accumulated sulfatide, suggesting that the kinetics of internalization and recycling appear to be altered indirectly.
KW - Animals
KW - Humans
KW - Gene Expression Regulation
KW - Mice
KW - Cricetinae
KW - Lysosomes enzymology
KW - Receptor, IGF Type 2 metabolism
KW - Cerebroside-Sulfatase deficiency
KW - Endocytosis
KW - Kidney cytology
KW - Kidney Tubules cytology
KW - Kinetics
KW - Receptors, Transferrin metabolism
KW - Sulfoglycosphingolipids metabolism
KW - Animals
KW - Humans
KW - Gene Expression Regulation
KW - Mice
KW - Cricetinae
KW - Lysosomes enzymology
KW - Receptor, IGF Type 2 metabolism
KW - Cerebroside-Sulfatase deficiency
KW - Endocytosis
KW - Kidney cytology
KW - Kidney Tubules cytology
KW - Kinetics
KW - Receptors, Transferrin metabolism
KW - Sulfoglycosphingolipids metabolism
M3 - SCORING: Zeitschriftenaufsatz
VL - 390
SP - 41
EP - 48
JO - BIOL CHEM
JF - BIOL CHEM
SN - 1431-6730
IS - 1
M1 - 1
ER -
