Molecular defect in processing α-fucosidase in fucosidosis

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

1980 • 809 citations

Fucosidosis

1969 • 190 citations

Practical enzymology of the sphingolipidoses

1977 • 183 citations

Human liver alpha-L-fucosidase. Purification, characterization, and immunochemical studies.

1975 • 128 citations

Glycosphingolipids in Cultured Human Skin Fibroblasts

1972 • 102 citations

Carboxyl-terminal proteolytic processing during biosynthesis of the lysosomal enzymes .beta.-glucuronidase and cathepsin D

1983 • 95 citations

Deficiency of α-L-Fucosidase

1972 • 79 citations

Structure of the accumulating oligosaccharide in fucosidosis.

1976 • 75 citations

Substrate specificity of human α-l-fucosidase

1977 • 48 citations

Opsismodysplasia: A new type of chondrodysplasia with predominant involvement of the bones of the hand and the vertebrae

1984 • 41 citations

Radioimmunoassay determinations of decreased amounts of α-L-fucosidase protein in fucosidosis

1982 • 27 citations

Purification and properties of two forms of human α-l-fucosidase

1980 • 25 citations

[58] Studies of lysosomal enzyme biosynthesis in cultured cells

1983 • 13 citations