Published in Proc Natl Acad Sci U S A on May 01, 1979
Chloroquine inhibits lysosomal enzyme pinocytosis and enhances lysosomal enzyme secretion by impairing receptor recycling. J Cell Biol (1980) 5.99
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Characterization of a membrane-associated receptor from bovine liver that binds phosphomannosyl residues of bovine testicular beta-galactosidase. Proc Natl Acad Sci U S A (1981) 2.32
Antibody to mannose 6-phosphate specific receptor induces receptor deficiency in human fibroblasts. EMBO J (1984) 2.11
Morphologic study of the internalization of a lysosomal enzyme by the mannose 6-phosphate receptor in cultured Chinese hamster ovary cells. Proc Natl Acad Sci U S A (1981) 1.81
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Synthesis of beta-hexosaminidase in cell-free translation and in intact fibroblasts: an insoluble precursor alpha chain in a rare form of Tay-Sachs disease. Proc Natl Acad Sci U S A (1982) 1.61
Mutant of Chinese hamster ovary cells with altered mannose 6-phosphate receptor activity is unable to synthesize mannosylphosphoryldolichol. Proc Natl Acad Sci U S A (1982) 1.58
Canine alpha-L-iduronidase deficiency. A model of mucopolysaccharidosis I. Am J Pathol (1982) 1.48
Lysosomal enzyme binding to mouse P388D1 macrophage membranes lacking the 215-kDa mannose 6-phosphate receptor: evidence for the existence of a second mannose 6-phosphate receptor. Proc Natl Acad Sci U S A (1985) 1.36
Bone marrow transplantation in canine mucopolysaccharidosis I. Effects within the central nervous system. J Clin Invest (1987) 1.26
Carbohydrate specificity of sea urchin sperm bindin: a cell surface lectin mediating sperm-egg adhesion. J Cell Biol (1982) 1.25
Biosynthesis and turnover of the phosphomannosyl receptor in human fibroblasts. Biochem J (1983) 1.21
A canine model of human alpha-L-iduronidase deficiency. Proc Natl Acad Sci U S A (1983) 1.20
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Biochemical and histopathological studies on patients with mucopolysaccharidoses, two of whom had been treated by fibroblast transplantation. J Clin Pathol (1983) 0.82
Regional assignment of the structural gene for human alpha-L-iduronidase. Proc Natl Acad Sci U S A (1984) 0.82
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Properties of the Syrian hamster phosphomannosyl receptor: an aggregate of low molecular weight proteins. Proc Natl Acad Sci U S A (1985) 0.79
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Determination of Biological Variance and Validation of a Fluorometric Assay for Measurement of α-l-Iduronidase Activity in Dried Blood Spots Samples: The First Experience in Iran. Indian J Clin Biochem (2014) 0.75
Incorporation of mannose 6-phosphate receptors into liposomes. Receptor topography and binding of alpha-mannosidase. Biochem J (1983) 0.75
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Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts. Proc Natl Acad Sci U S A (1977) 5.97
The isolation and properties of a rabbit liver binding protein specific for asialoglycoproteins. J Biol Chem (1974) 4.57
A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes. Biochem Biophys Res Commun (1972) 4.25
Recognition and receptor-mediated uptake of a lysosomal enzyme, alpha-l-iduronidase, by cultured human fibroblasts. Cell (1977) 3.79
Inherited disorders of lysosomal metabolism. Annu Rev Biochem (1975) 3.26
A recognition marker required for uptake of a lysosomal enzyme by cultured fibroblasts. Biochem Biophys Res Commun (1974) 2.83
Subcellular membrane topology and turnover of a rat hepatic binding protein specific for asialoglycoproteins. J Biol Chem (1979) 2.59
Evidence for lysosomal enzyme recognition by human fibroblasts via a phosphorylated carbohydrate moiety. Biochem J (1978) 2.46
Characterization of the low density lipoprotein receptor in membranes prepared from human fibroblasts. J Biol Chem (1978) 2.24
The transport of lysosomal enzymes. J Supramol Struct (1977) 2.22
Isolation and characterization of an avian hepatic binding protein specific for N-acetylglucosamine-terminated glycoproteins. J Biol Chem (1977) 2.18
Phosphohexosyl recognition is a general characteristic of pinocytosis of lysosomal glycosidases by human fibroblasts. J Clin Invest (1977) 2.18
Enzymic diagnosis of the genetic mucopolysaccharide storage disorders. Methods Enzymol (1978) 1.89
In vitro correction of deficient human fibroblasts by beta-glucuronidase from different human sources. Biochem Biophys Res Commun (1974) 1.81
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Intercellular contact and cell-surface galactosyl transferase activity (cell culture-mouse-radioautography-contact inhibition-cis-and trans-galactosylation). Proc Natl Acad Sci U S A (1972) 1.70
An alternative hypothesis of cellular transport of lysosomal enzymes in fibroblasts. Effect of inhibitors of lysosomal enzyme endocytosis on intra- and extra-cellular lysosomal enzyme activities. Biochem J (1978) 1.67
The role of glycosidically bound mannose in the assimilation of beta-galactosidase by generalized gangliosidosis fibroblasts. Biochem Biophys Res Commun (1976) 1.53
Human kidney alpha-L-iduronidase: purification and characterization. Arch Biochem Biophys (1978) 1.39
The fate of the plasma membrane during endocytosis. Biochem Soc Trans (1977) 1.22
The relationship of alpha-L-iduronidase and Hurler corrective factor. Arch Biochem Biophys (1976) 0.95
Synthetic substrates for alpha-L-iduronidase. Methods Enzymol (1978) 0.87
Biosynthesis of lysosomal enzymes in fibroblasts. Synthesis as precursors of higher molecular weight. J Biol Chem (1980) 6.60
Enzyme-replacement therapy in mucopolysaccharidosis I. N Engl J Med (2001) 4.28
A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes. Biochem Biophys Res Commun (1972) 4.25
Recognition and receptor-mediated uptake of a lysosomal enzyme, alpha-l-iduronidase, by cultured human fibroblasts. Cell (1977) 3.79
Two species of lysosomal organelles in cultured human fibroblasts. Cell (1979) 3.64
Biosynthesis of lysosomal enzymes in fibroblasts. Phosphorylation of mannose residues. J Biol Chem (1980) 3.48
Inherited disorders of lysosomal metabolism. Annu Rev Biochem (1975) 3.26
The defect in Hurler's and Hunter's syndromes: faulty degradation of mucopolysaccharide. Proc Natl Acad Sci U S A (1968) 2.89
A recognition marker required for uptake of a lysosomal enzyme by cultured fibroblasts. Biochem Biophys Res Commun (1974) 2.83
Biosynthesis and turnover of the mannose 6-phosphate receptor in cultured Chinese hamster ovary cells. J Biol Chem (1983) 2.73
Allelism, non-allelism, and genetic compounds among the mucopolysaccharidoses. Lancet (1972) 2.65
Hurler and Hunter syndromes: mutual correction of the defect in cultured fibroblasts. Science (1968) 2.40
The defect in the Hurler and Scheie syndromes: deficiency of -L-iduronidase. Proc Natl Acad Sci U S A (1972) 2.32
The transport of lysosomal enzymes. J Supramol Struct (1977) 2.22
The 193-kD vault protein, VPARP, is a novel poly(ADP-ribose) polymerase. J Cell Biol (1999) 2.14
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Human beta-hexosaminidase alpha chain: coding sequence and homology with the beta chain. Proc Natl Acad Sci U S A (1985) 2.03
Isolation and characterization of a novel ribonucleoprotein particle: large structures contain a single species of small RNA. J Cell Biol (1986) 1.98
Frequency of three Hex A mutant alleles among Jewish and non-Jewish carriers identified in a Tay-Sachs screening program. Am J Hum Genet (1990) 1.94
Enzymic diagnosis of the genetic mucopolysaccharide storage disorders. Methods Enzymol (1978) 1.89
The defect in the Hunter syndrome: deficiency of sulfoiduronate sulfatase. Proc Natl Acad Sci U S A (1973) 1.86
Molecular basis of adult-onset and chronic GM2 gangliosidoses in patients of Ashkenazi Jewish origin: substitution of serine for glycine at position 269 of the alpha-subunit of beta-hexosaminidase. Proc Natl Acad Sci U S A (1989) 1.86
Structural requirements for time-dependent inhibition of prostaglandin biosynthesis by anti-inflammatory drugs. Proc Natl Acad Sci U S A (1975) 1.86
Morphologic study of the internalization of a lysosomal enzyme by the mannose 6-phosphate receptor in cultured Chinese hamster ovary cells. Proc Natl Acad Sci U S A (1981) 1.81
Is there a mechanism for introducing acid hydrolases into liver lysosomes that is independent of mannose 6-phosphate recognition? Evidence from I-cell disease. Biochem Biophys Res Commun (1982) 1.79
Mouse model of Sanfilippo syndrome type B produced by targeted disruption of the gene encoding alpha-N-acetylglucosaminidase. Proc Natl Acad Sci U S A (1999) 1.79
Association of alpha- and beta-subunits during the biosynthesis of beta-hexosaminidase in cultured human fibroblasts. J Biol Chem (1984) 1.75
Vaults are up-regulated in multidrug-resistant cancer cell lines. J Biol Chem (1998) 1.74
Corrective factors for inborn errors of mucopolysaccharide metabolism. Ann N Y Acad Sci (1971) 1.65
Synthesis of beta-hexosaminidase in cell-free translation and in intact fibroblasts: an insoluble precursor alpha chain in a rare form of Tay-Sachs disease. Proc Natl Acad Sci U S A (1982) 1.61
Formation of galactolipids by chloroplasts. Biochem Biophys Res Commun (1964) 1.61
Vaults. III. Vault ribonucleoprotein particles open into flower-like structures with octagonal symmetry. J Cell Biol (1991) 1.57
Overexpression of the human lysosomal enzyme alpha-L-iduronidase in Chinese hamster ovary cells. Protein Expr Purif (1994) 1.55
Coated vesicles from rat liver and calf brain contain lysosomal enzymes bound to mannose 6-phosphate receptors. J Biol Chem (1983) 1.54
Assembly of vault-like particles in insect cells expressing only the major vault protein. J Biol Chem (2001) 1.54
Structure of the vault, a ubiquitous celular component. Structure (1999) 1.53
The defect in Hurler and Hunter syndromes. II. Deficiency of specific factors involved in mucopolysaccharide degradation. Proc Natl Acad Sci U S A (1969) 1.53
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Vaults and telomerase share a common subunit, TEP1. J Biol Chem (1999) 1.50
Evidence that vault ribonucleoprotein particles localize to the nuclear pore complex. J Cell Sci (1993) 1.49
Inhibition of receptor-mediated uptake of a lysosomal enzyme into fibroblasts by chloroquine, procaine and ammonia. Exp Cell Res (1979) 1.49
Enzyme replacement in a canine model of Hurler syndrome. Proc Natl Acad Sci U S A (1994) 1.49
Canine alpha-L-iduronidase deficiency. A model of mucopolysaccharidosis I. Am J Pathol (1982) 1.48
Long-term and high-dose trials of enzyme replacement therapy in the canine model of mucopolysaccharidosis I. Biochem Mol Med (1996) 1.48
Assay and purification of a solubilized membrane receptor that binds the lysosomal enzyme alpha-L-iduronidase. Arch Biochem Biophys (1982) 1.46
A -glucuronidase deficiency mucopolysaccharidosis: studies in cultured fibroblasts. Arch Biochem Biophys (1973) 1.45
Maturation of alpha-L-iduronidase in cultured human fibroblasts. J Biol Chem (1981) 1.44
The Hurler corrective factor. Purification and some properties. J Biol Chem (1971) 1.43
Vaults. II. Ribonucleoprotein structures are highly conserved among higher and lower eukaryotes. J Cell Biol (1990) 1.42
The Sanfilippo A corrective factor. Purification and mode of action. J Biol Chem (1972) 1.41
The molecular basis of Sanfilippo syndrome type B. Proc Natl Acad Sci U S A (1996) 1.41
Human kidney alpha-L-iduronidase: purification and characterization. Arch Biochem Biophys (1978) 1.39
Molecular analysis of Hurler syndrome in Druze and Muslim Arab patients in Israel: multiple allelic mutations of the IDUA gene in a small geographic area. Am J Hum Genet (1993) 1.38
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RNA location and modeling of a WD40 repeat domain within the vault. RNA (2000) 1.37
Incorporation of D-xylose-C14 into glycoprotein by particles from hen oviduct. Biochem Biophys Res Commun (1966) 1.37
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Architecture of the canine IDUA gene and mutation underlying canine mucopolysaccharidosis I. Genomics (1992) 1.28
Vault ribonucleoprotein particles from rat and bullfrog contain a related small RNA that is transcribed by RNA polymerase III. J Biol Chem (1993) 1.28
Coated vesicles from rat liver and calf brain contain cryptic mannose 6-phosphate receptors. J Biol Chem (1983) 1.26
Limited proteolysis of the beta-hexosaminidase precursor in a cell-free system. J Biol Chem (1981) 1.23
A canine model of human alpha-L-iduronidase deficiency. Proc Natl Acad Sci U S A (1983) 1.20
Glycosylation of serine residues by a uridine diphosphate-xylose: protein xylosyltransferase from mouse mastocytoma. Arch Biochem Biophys (1966) 1.20
Alpha-L-iduronidase activity in cultured skin fibroblasts and amniotic fluid cells. Arch Biochem Biophys (1973) 1.18
The Hunter syndrome in females: is there an autosomal recessive form of iduronate sulfatase deficiency? Am J Hum Genet (1977) 1.17
A frameshift mutation in a patient with Tay-Sachs disease causes premature termination and defective intracellular transport of the alpha-subunit of beta-hexosaminidase. J Biol Chem (1989) 1.17
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Cloning and characterization of cDNA encoding canine alpha-L-iduronidase. mRNA deficiency in mucopolysaccharidosis I dog. J Biol Chem (1992) 1.15
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A third mutation at the CpG dinucleotide of codon 504 and a silent mutation at codon 506 of the HEX A gene. Am J Hum Genet (1991) 1.14
Nonuniform deficiency of hexosaminidase A in tissues and fluids of two unrelated individuals. Pediatr Res (1982) 1.13
Simulation of genetic mucopolysaccharidoses in normal human fibroblasts by alteration of pH of the medium. Proc Natl Acad Sci U S A (1972) 1.13
Biochemical heterogeneity of the Sanfilippo syndrome: preliminary characterization of two deficient factors. Biochem Biophys Res Commun (1971) 1.12
Faulty association of alpha- and beta-subunits in some forms of beta-hexosaminidase A deficiency. J Biol Chem (1984) 1.12
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PCR-based cystic fibrosis (CF) carrier screening in a first-year medical student biochemistry laboratory. Am J Hum Genet (1993) 1.06
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