Coding of Two Sphingolipid Activator Proteins (SAP-1 and SAP-2) by Same Genetic Locus

AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2.

1978 • 274 citations

Gaucher's Disease: Deficiency of “cid”β-Glucosidase and Reconstitution of Enzyme Activity In Vitro

1971 • 257 citations

Enzymes of Lipid Metabolism II

1986 • 208 citations

Biosynthesis and molecular cloning of sulfated glycoprotein 1 secreted by rat Sertoli cells: sequence similarity with the 70-kilodalton precursor to sulfatide/GM1 activator

1988 • 166 citations

Immunochemical Characterization of Two Activator Proteins Stimulating Enzymic Sphingomyelin Degradation in vitro Absence of One of them in a Human Gaucher Disease Variant

1986 • 164 citations

Cerebroside sulfatase activator deficiency induced metachromatic leukodystrophy.

1981 • 146 citations

The Activator of Cerebroside Sulphatase. Purification from Human Liver and Identification as a Protein

1975 • 135 citations

Biological chemistry Hoppe-Seyler

1985 • 133 citations

Purification and characterization of a sulfated glycoprotein secreted by Sertoli cells

1986 • 119 citations

Activator protein required for the enzymatic hydrolysis of cerebroside sulfate. Deficiency in urine of patients affected with cerebroside sulfatase activator deficiency and identity with activators for the enzymatic hydrolysis of GM1 ganglioside and globotriaosylceramide.

1985 • 105 citations

Relationships of Human Protein Sequences to Those of Other Organisms

1986 • 90 citations

A protein activator of galactosylceramide β-galactosidase

1982 • 86 citations

Immunological evidence for deficiency in an activator protein for sulfatide sulfatase in a variant form of metachromatic leukodystrophy.

1983 • 81 citations

Biosynthesis of the sulfatide/GM1 activator protein (SAP-1) in control and mutant cultured skin fibroblasts

1986 • 73 citations

The gene coding for a sphingolipid activator protein, SAP-1, is on human chromosome 10

1985 • 61 citations

Synthesis and processing of sphingolipid activator protein-2 (SAP-2) in cultured human fibroblasts.

1986 • 59 citations

Mapping of the gene coding for the human G_M2 activator protein to chromosome 5

1985 • 58 citations

Mammalian Glucocerebrosidase: Implications for Gaucher’s Disease

1989 • 55 citations

Nucleotide sequence analysis of a proline-rich protein cDNA and peptide homologies of rat and human proline-rich proteins.

1984 • 53 citations

Complete Amino-Acid Sequence and Carbohydrate Content of the Naturally Occurring Glucosylceramide Activator Protein (A₁Activator) Absent from a New Human Gaucher Disease Variant

1987 • 52 citations

Nucleotide sequence of cloned cDNA for human sphingolipid activator protein 1 precursor.

1987 • 41 citations

Assignment of the gene for human sphingolipid activator protein-2 (SAP-2) to chromosome 10.

1985 • 41 citations

Molecular cloning of the sphingolipid activator protein — 1 (SAP - 1), the sulfatide sulfatase activator

1986 • 37 citations

Concentrations of an activator protein for sphingolipid hydrolysis in liver and brain samples from patients with lysosomal storage diseases.

1983 • 32 citations

Studies on the activation of the enzymatic hydrolysis of sphingomyelin liposomes

1984 • 32 citations

Studies on a sphingolipid activator protein ( SAP-2) in fibroblasts from patients with lysosomal storage diseases, including Niemann-Pick disease Type C

1985 • 31 citations

A triple-binding-domain model explains the specificity of the interaction of a sphingolipid activator protein (SAP-1) with sulphatide, GM1-ganglioside and globotriaosylceramide

1986 • 25 citations

Subcellular localization of the heat-stable glucocerebrosidase activator substance in gaucher spleen

1978 • 18 citations

Immunocytochemical localization of sphingolipid activator protein-1, the sulfatide/GM1 ganglioside activator, to lysosomes in human liver and colon

1986 • 18 citations

Regional localization of the gene coding for sphingolipid activator protein SAP-1 on human chromosome 10

1987 • 18 citations

High-resolution proton nuclear magnetic resonance studies of the glucocerebrosidase activator protein from Gaucher spleen

1985 • 13 citations

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