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

Comparative Aspects of Glycoprotein Structure

1976 • 767 citations

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

1972 • 446 citations

A recognition marker required for uptake of a lysosomal enzyme by cultured fibroblasts

1974 • 281 citations

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

1977 • 181 citations

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

1977 • 158 citations

Acid phosphatase of osteoclasts demonstrated by electron microscopic histochemistry

1971 • 134 citations

Australia Antigen in Volunteer and Paid Blood Donors

1971 • 123 citations

The existence of a second route for the transfer of certain glycoproteins from the circulation into the liver

1976 • 112 citations

Recognition of lysosomal glycosidases in vivo inhibited by modified glycoproteins

1976 • 102 citations

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

1976 • 101 citations

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

1975 • 92 citations

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

1976 • 66 citations

Structure of the three major sialyl-oligosaccharides excreted in the urine of five patients with three distinct inborn diseases: “I cell disease” and two new types of mucolipidosis

1976 • 65 citations

Sandhoff Disease: Defective Glycosaminoglycan Catabolism in Cultured Fibroblasts and Its Correction by β‐N‐Acetylhexosaminidase

1974 • 61 citations

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

1976 • 43 citations