Identification of the binding and activating sites of the sphingolipid activator protein, saposin C, with glucocerebrosidase

Protein Science

Volumn 4, Issue 4, 1995, Pages 756-764

  Weiler, Solly ^a,d   Tomich, John M ^a,e   Kishimoto, Yasuo ^b   O'Brien, John S ^b   Barranger, John A ^c  

^a UNIVERSITY OF SOUTHERN CALIFORNIA   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c UNIVERSITY OF PITTSBURGH   (United States)

^d NATIONAL HEART LUNG AND BLOOD INSTITUTE   (United States)

^e KANSAS STATE UNIVERSITY   (United States)

Author keywords

Ceredase; deglycosylation; enzyme activation; Gaucher disease; glucocerebrosidase; saposin A; saposin C; sphingolipid activator proteins; synthetic peptides

Indexed keywords

ALGLUCERASE; GLUCOSYLCERAMIDASE; SPHINGOLIPID ACTIVATOR PROTEIN; SYNTHETIC PEPTIDE;

ARTICLE; BINDING SITE; DEGLYCOSYLATION; ENZYME ACTIVATION; ENZYME ACTIVE SITE; ENZYME ACTIVITY; GAUCHER DISEASE; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN BINDING;

AMINO ACID SEQUENCE; BINDING SITES; BINDING, COMPETITIVE; CONSERVED SEQUENCE; ENZYME ACTIVATION; GLUCOSYLCERAMIDASE; GLYCOPROTEINS; HUMAN; LYSOSOMES; MOLECULAR SEQUENCE DATA; PEPTIDES; PROTEIN STRUCTURE, SECONDARY; SPHINGOLIPIDS; SUPPORT, NON-U.S. GOV'T; SUPPORT, U.S. GOV'T, P.H.S.;

EID: 0028945615     PISSN: 09618368     EISSN: 1469896X     Source Type: Journal    
DOI: 10.1002/pro.5560040415     Document Type: Article

References (39)

1. Relationship between the two immunologically distinguishable forms of glucocerebrosidase in tissue extracts
2. A procedure for the rapid purification in high yield of human glucocerebrosidase using immunoaffinity chromatography with monoclonal antibodies
3. + enriched cord blood and Gaucher bone marrow cells
4. Glucosylceramide lipidoses: Gaucher disease
5. Characterization of the phospholipid requirement of rat liver β‐glucosidase
6. Activators of spleen glucocerebrosidase from controls and patients with various forms of Gaucher's disease
7. Comparison of N‐acylphosphatidylethanolamines with different N‐acyl groups as activators of glucocerebrosidase in various forms of Gaucher's disease
8. β‐Glucosidase activator protein from bovine spleen (“coglucosidase”)
9. Gaucher disease: New molecular approaches to diagnosis and treatment
10. Metabolism of glucocerebrosides II. Evidence of an enzymatic deficiency in Gaucher's disease
11. Immunochemical characterization of two activator proteins stimulating enzymic sphingomyelin degradation in vitro. Absence of one of them in a human Gaucher disease variant
12. Activator protein deficient Gaucher's disease. A second patient with the newly identified lipid storage disorder
13. Solubilization of glucocerebrosidase from human placenta and demonstration of a phospholipid requirement for activity
14. Human acid β‐glucosidase. Use of inhibitory and activating monoclonal antibodies to investigate the enzyme's catalytic mechanism and saposin C and saposin A binding sites
15. Synthesis and processing of sphingolipid activator protein‐2 (SAP‐2) in cultured human fibroblasts
16. Uptake and distribution of placental glucocerebrosidase in rat hepatic cells and effects of sequential deglycosylation
17. Sphingolipid activator protein deficiency in a 16‐week old atypical Gaucher disease patient and his fetal sibling: Biochemical signs of combined sphingolipidoses
18. Glucocerebrosidase: Reconstitution from macromolecular components depends on acidic phospholipids
19. Gaucher's disease: Deficiency of “acid” β‐glucosidase and reconstitution of enzyme activity in vitro
20. Glucocerebrosidase: Reconstitution of activity from macromolecular components
21. Glucocerebrosidase: Stoichiometry of association between effector and catalytic proteins
22. Solid phase peptide synthesis. I. The synthesis of a tetrapeptide
23. Interaction of saposins, acidic lipids and glucosylceramidase
24. Saposin A: Second cerebrosidase activator protein
25. Immunoelectron‐microscopic localization of mannose‐terminal glucocerebrosidase in lysosomes of rat liver Kupffer cells
26. Transduction of G‐CSF primed peripheral blood CD34+ cells with the glucocerebrosidase
27. Saposin proteins: Structure, function and role in human lysosomal storage disorders
28. Efficient transfer and sustained high expression of the human glucocerebrosidase gene in mice
29. Immunocytochemical localization of sphingolipid activator protein 2 (SAP‐2) in normal and SAP‐deficient fibroblasts
30. A deficiency of glucocerebrosidase in Gaucher's disease
31. Further studies on the activation of glucocerebrosidase by a heat‐stable factor from Gaucher spleen
32. The cohydrolases for cerebroside β‐glucosidase
33. Quantitative monitoring of solid‐phase peptide synthesis by the ninhydrin reaction
34. Simultaneous deficiency of sphingolipid activator proteins 1 and 2 is caused by a mutation in the initiation codon of their common gene
35. Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease
36. High‐resolution proton nuclear magnetic resonance studies of the glucocerebrosidase activator protein from Gaucher spleen
37. Porcine cerebroside sulfate activator: Further structural characterization and disulfide identification
38. Synthesis and characterization of a bioactive 82‐residue sphingolipid activator protein