Mucolipidosis III β-N-acetyl-<scp>d</scp>-hexosaminidase A. Purification and properties

Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4

1970 • 253,849 citations

Short-Term Effects of Nose-Only Cigarette Smoke Exposure on Glutathione Redox Homeostasis, Cytochrome P450 1A1/2 and Respiratory Enzyme Activities in Mice Tissues

2013 • 47,043 citations

Electrophoretic analysis of the major polypeptides of the human erythrocyte membrane

1971 • 8,619 citations

Biosynthesis of lysosomal enzymes in fibroblasts. Synthesis as precursors of higher molecular weight.

1980 • 809 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

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

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

1980 • 225 citations

I-Cell Disease: Biochemical Studies

1972 • 215 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

Phosphorylated oligosaccharides in lysosomal enzymes: identification of alpha-N-acetylglucosamine(1)phospho(6)mannose diester groups.

1980 • 131 citations

Studies on Human β-d-N-Acetylhexosaminidases

1974 • 118 citations

The subunit and polypeptide structure of hexosaminidases from human placenta

1980 • 115 citations

Isolation and Characterization of Phosphorylated Oligosaccharides from alpha-N-Acetylglucosaminidase that Are Recognized by Cell-Surface Receptors

1979 • 101 citations

Oligosaccharides in Lysosomal Enzymes

1981 • 97 citations

Specificity of Human Liver Hexosaminidases A and B against Glycosphingolipids G_M2and G_A2. Purification of the Enzymes by Affinity Chromatography Employing Specific Elution

1977 • 91 citations

I-Cell disease: Activities of lysosomal enzymes toward natural and synthetic substrates

1976 • 82 citations

Structure and function of gangliosides

1981 • 79 citations

Limited proteolysis of the beta-hexosaminidase precursor in a cell-free system.

1981 • 64 citations

Leroy's l-cell disease: markedly increased activity of plasma acid hydrolases.

1974 • 55 citations

Purification of normal human urinary N-acetyl-β-hexosaminidase A by affinity chromatography

1975 • 34 citations

Purification and characterization of I-cell disease alpha-L-fucosidase.

1980 • 33 citations

Mucolipidosis Type III

1978 • 24 citations

Carbohydrate composition of human placental N-acetylhexosaminidase A and B

1979 • 23 citations

Effect of tunicamycin and cycloheximide on the secretion of acid hydrolases from I-cell cultured fibroblasts

1980 • 22 citations

The effect of monensin on β-hexosaminidase transport in normal and I-cell fibroblasts

1980 • 20 citations

Defective phosphorylation and processing of β-hexosaminidase by intact cultured fibroblasts from patients with mucolipidosis III

1982 • 16 citations

A study of highly purified mucolipidosis III urinary N-acetyl-β-D-hexosaminidase B

1982 • 8 citations

Purification and properties of N-acetyl-β-hexosaminidases from human urine

1978 • 7 citations