Effect of the co-existence of galactosyl and phosphomannosyl residues on β-hexosaminidase on the processing and transport of the enzyme in mucolipidosis I fibroblasts

A Rapid and Sensitive Method for the Quantitation of Microgram Quantities of Protein Utilizing the Principle of Protein-Dye Binding

1976 • 225,752 citations

Specificity of Twelve Lectins Towards Oligosaccharides and Glycopeptides Related to N‐Glycosylproteins

1981 • 773 citations

Chloroquine inhibits lysosomal enzyme pinocytosis and enhances lysosomal enzyme secretion by impairing receptor recycling.

1980 • 743 citations

A hypothesis for I-cell disease: Defective hydrolases that do not enter lysosomes

1972 • 446 citations

Biosynthesis of lysosomal enzymes in fibroblasts. Phosphorylation of mannose residues.

1980 • 404 citations

Recognition and receptor-mediated uptake of a lysosomal enzyme, α-l-iduronidase, by cultured human fibroblasts

1977 • 354 citations

Fibroblasts from patients with I-cell disease and pseudo-Hurler polydystrophy are deficient in uridine 5'-diphosphate-N-acetylglucosamine: glycoprotein N-acetylglucosaminylphosphotransferase activity.

1981 • 323 citations

Enzymatic phosphorylation of lysosomal enzymes in the presence of UDP-N-acetylglucosamine. Absence of the activity in l-cell fibroblasts

1981 • 299 citations

The spectrum of anionic oligosaccharides released by endo-beta-N-acetylglucosaminidase H from glycoproteins. Structural studies and interactions with the phosphomannosyl receptor.

1983 • 244 citations

Phosphomannosyl-enzyme receptors in rat liver. Subcellular distribution and role in intracellular transport of lysosomal enzymes.

1980 • 231 citations

UDP-N-acetylglucosamine:glycoprotein N-acetylglucosamine-1-phosphotransferase. Proposed enzyme for the phosphorylation of the high mannose oligosaccharide units of lysosomal enzymes.

1981 • 229 citations

Biosynthetic intermediates of beta-glucuronidase contain high mannose oligosaccharides with blocked phosphate residues.

1980 • 225 citations

Evidence for lysosomal enzyme recognition by human fibroblasts via a phosphorylated carbohydrate moiety

1978 • 185 citations

Phosphohexosyl recognition is a general characteristic of pinocytosis of lysosomal glycosidases by human fibroblasts.

1977 • 181 citations

Identification of a rat liver alpha-N-acetylglucosaminyl phosphodiesterase capable of removing “blocking” alpha-N-acetylglucosamine residues from phosphorylated high mannose oligosaccharides of lysosomal enzymes.

1980 • 174 citations

Is there a mechanism for introducing acid hydrolases into liver lysosomes that is independent of mannose 6-phosphate recognition? evidence from I-cell disease

1982 • 169 citations

Mucolipidosis I: Increased sialic acid content and deficiency of an α-N-acetylneuraminidase in cultured fibroblasts

1977 • 158 citations

Processing of the phosphorylated recognition marker in lysosomal enzymes. Characterization and partial purification of a microsomal alpha-N-acetylglucosaminyl phosphodiesterase.

1981 • 154 citations

Deficiency of UDP-N-acetylglucosamine: Lysosomal enzyme N-acetylglucosamine-1-phosphotransferase in organs of I-cell patients

1982 • 147 citations

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

1978 • 111 citations

Abnormal lysosomal hydrolases excreted by cultured fibroblasts in I-cell disease (mucolipidosis II)

1975 • 92 citations

Subcellular location of two enzymes involved in the synthesis of phosphorylated recognition markers in lysosomal enzymes.

1981 • 85 citations

I-cell disease: Deficiency of extracellular hydrolase phosphorylation

1979 • 82 citations

Excretion-Reuptake Route of β-Hexosaminidase in Normal and I-Cell Disease Cultured Fibroblasts

1979 • 76 citations

Properties of N-acetyl-beta-D-hexosaminidase from isolated normal and I-cell lysosomes.

1981 • 49 citations

Demonstration of the heterozygous state for I-cell disease and pseudo-Hurler polydystrophy by assay of N-acetylglucosaminylphosphotransferase in white blood cells and fibroblasts.

1982 • 44 citations

The effect of chloroquine on the distribution of newly synthesized and old β-hexosaminidase in fibroblasts

1982 • 12 citations

Electrophoretic analysis of glycoprotein enzymes in the sialidoses and mucolipidoses

1981 • 10 citations