Lack of proteolytic processing of α‐L‐fucosidase in human skin fibroblasts

PROTEIN MEASUREMENT WITH THE FOLIN PHENOL REAGENT

1951 • 319,299 citations

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

1980 • 809 citations

Pulmonary Diseases and Disorders.

1988 • 553 citations

Trafficking of lysosomal enzymes in normal and disease states.

1986 • 445 citations

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

1980 • 404 citations

Molecular defect in combined beta-galactosidase and neuraminidase deficiency in man.

1982 • 364 citations

Biosynthesis and transport of cathepsin D in cultured human fibroblasts.

1983 • 235 citations

Identification and characterization of cells deficient in the mannose 6-phosphate receptor: evidence for an alternate pathway for lysosomal enzyme targeting.

1983 • 186 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

Processing of human cathepsin D in lysosomes in vitro.

1985 • 159 citations

Synthesis of beta-hexosaminidase in cell-free translation and in intact fibroblasts: an insoluble precursor alpha chain in a rare form of Tay-Sachs disease.

1982 • 113 citations

The interaction of phosphorylated oligosaccharides and lysosomal enzymes with bovine liver cation-dependent mannose 6-phosphate receptor.

1987 • 106 citations

Synthesis and processing of alpha-galactosidase A in human fibroblasts. Evidence for different mutations in Fabry disease.

1987 • 85 citations

Cathepsin D and beta-hexosaminidase synthesized in the presence of 1-deoxynojirimycin accumulate in the endoplasmic reticulum.

1984 • 84 citations

Chromogenic Immuno detection of Human Serum Albumin and α-<scp>l</scp>-Fucosidase Clones in a Human Hepatoma cDNA Expression Library

1984 • 74 citations

[58] Glycosylation inhibitors for n-linked glycoproteins

1987 • 71 citations

Turnover of two lysosomal enzymes in macrophages.

1981 • 62 citations

Biosynthesis and maturation of arylsulfatase B in normal and mutant cultured human fibroblasts.

1983 • 62 citations

Synthesis and transport of lysosomal acid phosphatase in normal and I-cell fibroblasts.

1985 • 60 citations

Altered Fucosylation of Membrane Glycoproteins from Cystic Fibrosis Fibroblasts1

1985 • 41 citations

A glycoprotein from neurites of differentiated neuroblastoma cells.

1980 • 37 citations

Transit of alpha-mannosidase during its maturation in Dictyostelium discoideum.

1985 • 35 citations

Targeting of beta-glucuronidase to lysosomes in mannose 6-phosphate receptor-deficient MOPC 315 cells.

1984 • 33 citations

Abnormal distribution of α-L-fucosidase in cystic fibrosis: Increased activity in skin fibroblasts

1977 • 27 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

Molecular defect in processing α-fucosidase in fucosidosis

1985 • 24 citations

Lysosomal enzyme trafficking in mannose 6-phosphate receptor-positive mouse L-cells: demonstration of a steady state accumulation of phosphorylated acid hydrolases.

1986 • 24 citations

Altered isoenzyme patterns of liver α‐L‐fucosidase in cystic fibrosis

1976 • 20 citations

Biosynthesis of α-galactosidase A in cultured chang liver cells

1983 • 20 citations

Abnormal distribution of α-l-fucosidase in cystic fibrosis: Decreased activity in serum

1979 • 17 citations

A simple, rapid method for detecting mycoplasma contamination

1976 • 16 citations

Differential Concanavalin A binding of cystic fibrosis and normal liver α-L-fucosidase

1979 • 8 citations