Oligosaccharide and Ganglioside Neuraminidase Activities of Mucolipidosis I (Sialidosis) and Mucolipidosis II (I‐Cell Disease) Fibroblasts

PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT

1951 • 319,299 citations

The Thiobarbituric Acid Assay of Sialic Acids

1959 • 6,482 citations

Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts.

1977 • 580 citations

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

1977 • 354 citations

Ganglioside structures and distribution: Are they localized at the nerve ending?

1978 • 307 citations

Digestive Activity of Lysosomes

1968 • 219 citations

I-Cell Disease: Biochemical Studies

1972 • 215 citations

The transport of lysosomal enzymes

1977 • 214 citations

Macular cherry-red spots and myoclonus with dementia: Coexistent neuraminidase and β-galactosidase deficiencies

1978 • 203 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

Substrate specificity of neuraminidases

1973 • 174 citations

I-cell disease: A clinical picture

1971 • 166 citations

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

1977 • 158 citations

CORRECTIVE FACTORS FOR INBORN ERRORS OF MUCOPOLYSACCHARIDE METABOLISM

1971 • 156 citations

Structure of Nine Sialyl-Oligosaccharides Accumulated in Urine of Eleven Patients with Three Different Types of Sialidosis. Mucolipidosis II and Two New Types of Mucolipidosis

1977 • 140 citations

I-cell disease

1984 • 130 citations

Chemical characterization and subunit structure of human N-acetylhexosaminidases A and B

1976 • 128 citations

Enzymatic hydrolysis of sphingolipids VII. Hydrolysis of gangliosides by a neuraminidase from calf brain

1968 • 109 citations

Clinical, biochemical, and ultrastructural studies in a case of chondrodystrophy presenting the I-cell phenotype in tissue culture

1971 • 107 citations

[121] Corrective factors for inborn errors of mucopolysaccharide metabolism

1972 • 104 citations

Studies on the Soluble and Lysosomal Neuraminidases of Rat Liver

1970 • 102 citations

Mucolipidosis I — A sialidosis

1977 • 102 citations

Isolated acid neuraminidase deficiency: A distinct lysosomal storage disease

1977 • 101 citations

Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease (mucolipidosis II) fibroblasts

1976 • 101 citations

On the Lysosomal Occurrence and the Properties of the Neuraminidase of Rat Liver and of Ehrlich Ascites Tumor Cells

1968 • 98 citations

Neuraminidase deficiency in the cherry red spot-myoclonus syndrome

1977 • 93 citations

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

1975 • 92 citations

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

1976 • 82 citations

Sialidase (α‐N‐acetyl neuraminidase) deficiency: the enzyme defect in an adult with macular cherry‐red spots and myoclonus without dementia

1978 • 76 citations

Occurrence of disialosyl groups in glycoproteins

1977 • 68 citations

The role of glycosidically bound mannose in the assimilation of β-galactosidase by generalized gangliosidosis fibroblasts

1976 • 66 citations

Glycosphingolipids in Cultured Human Skin Fibroblasts

1972 • 65 citations

Lipomucopolysaccharidose

1968 • 60 citations

Biochemical basis of six different types of sialidosis

1978 • 50 citations

Progress in Medical Genetics

1975 • 44 citations

Deficit in neuraminidase associated with mucolipidosis II (I-cell disease).

1976 • 43 citations

Structures of sialyl‐oligosaccharides excreted in the urine of a patient with mucolipidosis I

1977 • 41 citations

Isolation and characterization of a bacteriochlorophyll‐associated chromatophore protein from Rhodospirillum rubrum G‐9

1977 • 39 citations

I-cell disease Desialylation of β-hexosaminidase and its effect on uptake by fibroblasts

1978 • 22 citations

KERATAN SULFATE OF CARTILAGE AND NUCLEUS PULPOSUS

1975 • 4 citations