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"I-cell" disease: leakage of lysosomal enzymes into extracellular fluids.
N Engl J Med. 1971 Nov 4;285(19):1090-1
PMID: 4328711
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Multiple lysosomal enzyme deficiency due to enzyme leakage?
N Engl J Med. 1971 Jan 14;284(2):109-10
PMID: 4249768
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Clinical, biochemical, and ultrastructural studies in a case of chondrodystrophy presenting the I-cell phenotype in tissue culture.
J Pediatr. 1971 Sep;79(3):366-78
PMID: 4327937
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Recognition and receptor-mediated uptake of a lysosomal enzyme, alpha-l-iduronidase, by cultured human fibroblasts.
Cell. 1977 Nov;12(3):619-27
PMID: 922886
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I-Cell disease: isoelectric focusing, concanavalin A-Sepharose 4B binding and kinetic properties of human liver acid beta-D-galactosidases.
Biochim Biophys Acta. 1978 Jan 12;522(1):174-86
PMID: 413580
-
Catabolism of desialylated ceruloplasmin in the liver.
J Biol Chem. 1970 Nov 10;245(21):5833-7
PMID: 5472375
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Enzymatic phosphorylation of lysosomal enzymes in the presence of UDP-N-acetylglucosamine. Absence of the activity in I-cell fibroblasts.
Biochem Biophys Res Commun. 1981 Feb 12;98(3):761-7
PMID: 6452876
-
Effects of tunicamycin on the biosynthesis of procollagen by human fibroblasts.
J Biol Chem. 1980 Jan 10;255(1):121-8
PMID: 7350144
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Abnormal lysosomal hydrolases excreted by cultured fibroblasts in I-cell disease (mucolipidosis II).
Biochem Biophys Res Commun. 1975 Dec 1;67(3):956-64
PMID: 1201084
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Phosphohexosyl recognition is a general characteristic of pinocytosis of lysosomal glycosidases by human fibroblasts.
J Clin Invest. 1977 Nov;60(5):1088-93
PMID: 908752
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Mutant enzymatic and cytological phenotypes in cultured human fibroblasts.
Science. 1967 Aug 18;157(3790):804-6
PMID: 17842782
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Autolysis of glycoproteins in rat kidney lysosomes in vitro. Effects on the isoelectric focusing behaviour of glycoproteins, arylsulphatase and beta-glucuronidase.
Biochem J. 1974 Aug;141(2):527-35
PMID: 4455220
-
Biosynthesis of lysosomal enzymes in fibroblasts. Phosphorylation of mannose residues.
J Biol Chem. 1980 May 25;255(10):4946-50
PMID: 6989822
-
Multiple molecular forms of arylsulfatase A in different forms of metachromatic leukodystrophy (MLD).
Neurology. 1979 Jan;29(1):16-20
PMID: 34126
-
Studies of the mechanism of tunicamycin in hibition of IgA and IgE secretion by plasma cells.
J Biol Chem. 1977 Jun 25;252(12):4402-8
PMID: 325006
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Enveloped virus acquires membrane defect when passaged in fibroblasts from I-cell disease patients.
Proc Natl Acad Sci U S A. 1976 Jul;73(7):2443-7
PMID: 821054
-
Globoid cell (Krabbe's) leukodystrophy: heterozygote detection in cultured skin fibroblasts.
Am J Hum Genet. 1973 Nov;25(6):604-9
PMID: 4773478
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Processing of high mannose oligosaccharides to form complex type oligosaccharides on the newly synthesized polypeptides of the vesicular stomatitis virus G protein and the IgG heavy chain.
J Biol Chem. 1978 Feb 10;253(3):716-22
PMID: 202596
-
Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts.
Proc Natl Acad Sci U S A. 1977 May;74(5):2026-30
PMID: 266721
-
The synthesis of complex-type oligosaccharides. III. Identification of an alpha-D-mannosidase activity involved in a late stage of processing of complex-type oligosaccharides.
J Biol Chem. 1978 Nov 10;253(21):7779-86
PMID: 212436
-
Multiple molecular forms of certain lysosomal enzymes separated by isoelectric focusing on cellulose acetate membranes.
Clin Chim Acta. 1978 Oct 2;89(1):145-55
PMID: 30549
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The binding of desialylated glycoproteins by plasma membranes of rat liver.
J Biol Chem. 1971 Aug 10;246(15):4825-33
PMID: 4327329
-
Isoelectric focusing of acid hydrolases in human liver and serum. Findings in sera from one patient with I-cell disease phenotype.
Clin Chim Acta. 1978 Sep 15;88(3):441-8
PMID: 699337
-
I-cell disease: deficiency of extracellular hydrolase phosphorylation.
Biochem Biophys Res Commun. 1979 Dec 14;91(3):976-81
PMID: 526296
-
Protein measurement with the Folin phenol reagent.
J Biol Chem. 1951 Nov;193(1):265-75
PMID: 14907713
-
Physicochemical modifications of lysosomal hydrolases during intracellular transport.
Biochem J. 1973 Feb;132(2):267-82
PMID: 4725040
-
Biosynthetic intermediates of beta-glucuronidase contain high mannose oligosaccharides with blocked phosphate residues.
J Biol Chem. 1980 Jul 25;255(14):6633-9
PMID: 7391040
-
Effect of tunicamycin on the biosynthesis of the major human red cell sialoglycoprotein, glycophorin A, in the leukemia cell line K562.
J Biol Chem. 1980 Mar 10;255(5):2169-75
PMID: 7354085
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The synthesis of complex-type oligosaccharides. II. Characterization of the processing intermediates in the synthesis of the complex oligosaccharide units of the vesicular stomatitis virus G protein.
J Biol Chem. 1978 Nov 10;253(21):7771-8
PMID: 212435
-
A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.
Biochem Biophys Res Commun. 1972 Nov 15;49(4):992-9
PMID: 4345092
-
The synthesis of complex-type oligosaccharides. I. Structure of the lipid-linked oligosaccharide precursor of the complex-type oligosaccharides of the vesicular stomatitis virus G protein.
J Biol Chem. 1978 Nov 10;253(21):7762-70
PMID: 212434