ZIB

1

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Acyl-CoA: dihydroxyacetone phosphate acyltransferase in human skin fibroblasts: study of its properties using a new assay method

Schutgens, R.B.H. ; Romeyn, G.-J. ; Ofman, R. ; [et al.]

Amsterdam : Elsevier

Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 879 (1986), S. 286-291

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ISSN: 0005-2760

Keywords: (Human skin fibroblast) ; Acyl transferase ; Etherphospholipid ; Peroxisome ; Plasmalogen ; Zellweger syndrome

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Chemistry and Pharmacology , Medicine , Physics

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2760(86)90217-1

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2

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Genetic relationship between lysosomal and lamellar body-specific α-glucosidases in human lung

de Vries, A.C.J. ; Schram, A.W. ; Tager, J.M. ; [et al.]

Amsterdam : Elsevier

Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 878 (1986), S. 288-291

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ISSN: 0005-2760

Keywords: (Human lung) ; Glycogenosis type II ; Lamellar body ; Pulmonary surfactant ; α-Glucosidase

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Chemistry and Pharmacology , Medicine , Physics

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2760(86)90159-1

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3

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An improved procedure for the isolation of lamellar bodies from human lung. Lamellar bodies free of lysosomes contain a spectrum of lysosomal-type hydrolases

de Vries, A.C.J. ; Schram, A. ; van den Berg, M. ; [et al.]

Amsterdam : Elsevier

Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 922 (1987), S. 259-269

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ISSN: 0005-2760

Keywords: (Human lung) ; Lamellar body ; Lysosomal enzyme ; Pulmonary surfactant ; α-Glucosidase

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Chemistry and Pharmacology , Medicine , Physics

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2760(87)90048-8

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4

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Comparison of the properties of a soluble form of glucocerebrosidase from human urine with those of the membrane-associated tissue enzyme

Aerts, J.M.F.G. ; Donker-Koopman, W.E. ; Koot, M. ; [et al.]

Amsterdam : Elsevier

Biochimica et Biophysica Acta (BBA)/Biomembranes 863 (1986), S. 63-70

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ISSN: 0005-2736

Keywords: (Human urine) ; Gaucher disease ; Glucocerebrosidase ; Glycosphingolipidosis ; Lysosomal enzyme ; β-Glucosidase

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Chemistry and Pharmacology , Medicine , Physics

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2736(86)90387-1

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5

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A specific acid α-glucosidase in lamellar bodies of the human lung

de Vries, A.C.J. ; Schram, A. ; Tager, J.M. ; [et al.]

Amsterdam : Elsevier

Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 837 (1985), S. 230-238

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ISSN: 0005-2760

Keywords: (Human lung) ; Lamellar body ; Lung surfactant ; Lysosomal enzyme ; α-Glucosidase

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Chemistry and Pharmacology , Medicine , Physics

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2760(85)90046-3

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6

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Acid sphingomyelinase from human urine: purification and characterization

Quintern, L.E. ; Weitz, G. ; Nehrkorn, H. ; [et al.]

Amsterdam : Elsevier

Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 922 (1987), S. 323-336

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ISSN: 0005-2760

Keywords: (Human urine) ; Enzyme purification ; Lysosomal enzyme ; Niemann-Pick disease ; Sphingomyelinase

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Chemistry and Pharmacology , Medicine , Physics

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2760(87)90055-5

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7

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The cerebro-hepato-renal (Zellweger) syndrome. Impaired de novo biosynthesis of plasmalogens in cultured skin fibroblasts

Schrakamp, G. ; Schutgens, R.B.H. ; Wanders, R.J.A. ; [et al.]

Amsterdam : Elsevier

Biochimica et Biophysica Acta (BBA)/Lipids and Lipid Metabolism 833 (1985), S. 170-174

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ISSN: 0005-2760

Keywords: (Amniotic fluid cell ; Ether lipid synthesis ; Inborn error ; Plasmalogen ; Skin fibroblast) ; Zellweger syndrome

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Chemistry and Pharmacology , Medicine , Physics

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2760(85)90266-8

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8

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Use of immobilized antibodies in investigating acid α-glucosidase in urine in relation to Pompe's disease

Schram, A. ; Brouwer-Kelder, B. ; Donker-Koopman, W.E. ; [et al.]

Amsterdam : Elsevier

BBA - Enzymology 567 (1979), S. 370-383

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ISSN: 0005-2744

Keywords: Acid maltase ; Glycogenosis ; Lysosomal enzyme ; Pompe's disease ; α-Glucosidase

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0005-2744(79)90123-2

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9

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Phenotypic heterogeneity in cultured skin fibroblasts from patients with disorders of peroxisome biogenesis belonging to the same complementation group

Wiemer, E.A.C. ; Out, M. ; Schelen, A. ; [et al.]

Amsterdam : Elsevier

Biochimica et Biophysica Acta (BBA)/Molecular Basis of Disease 1097 (1991), S. 232-237

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ISSN: 0925-4439

Keywords: Catalase ; Peroxisomal biogenesis ; Peroxisomal β-oxidation ; Zellweger syndrome

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Biology , Chemistry and Pharmacology , Medicine , Physics

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0925-4439(91)90041-7

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10

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Peroxisomal very long-chain fatty acid β-oxidation in human skin fibroblasts: activity in Zellweger syndrome and other peroxisomal disorders

Wanders, R.J.A. ; van Roermund, C.W.T. ; van Wijland, M.J.A. ; [et al.]

Amsterdam : Elsevier

Clinica Chimica Acta 166 (1987), S. 255-263

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ISSN: 0009-8981

Keywords: Adrenoleukodystrophy ; Peroxisomal β-oxidation ; Peroxisome ; Refsum disease ; Very long-chain fatty acid ; Zellweger syndrome

Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002

Topics: Medicine

Type of Medium: Electronic Resource

URL: http://linkinghub.elsevier.com/retrieve/pii/0009-8981(87)90428-1

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