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Literature DB >> 1070017

Enzyme replacement therapy in Gaucher's disease: a rapid, high-yield method for purification of glucocerebrosidase.

Abstract

Gaucher's disease is caused by a deficiency of the lysosomal enzyme glucocerebrosidase (glucosylceramidase; D-glucosyl-N-acylsphingosine glucohydrolase, EC 3.2.1.45); this disorder has been a leading candidate for enzyme replacement trials. A rapid, high-yield method for purification of glucocerebrosidase has been developed. Detergent extraction of human placenta was followed by salt fractionation, concanavalin A-Sepharose chromatography, organic solvent precipitation, and affinity chromatography on phosphatidylserine-agarose; the total yield is 60% with 6000-fold purification. Purified glucocerebrosidase has been administered intravenously to a volunteer Gaucher's patient on two separate occasions. For the first injection, the enzyme was entrapped in resealed erythrocytes; for the second injection, the enzyme was given without any carrier. The enzyme infusions caused no untoward effects.

Entities: Chemical Disease Gene Species

Mesh：

Substances：

Year: 1976 PMID： 1070017 PMCID： PMC431591 DOI： 10.1073/pnas.73.12.4672

Source DB: PubMed Journal: Proc Natl Acad Sci U S A ISSN： 0027-8424 Impact factor: 11.205

11 in total

1. A reinvestigation of the cerebroside deposited in Gaucher's disease.

Authors: A ROSENBERG; E CHARGAFF
Journal: J Biol Chem Date: 1958-12 Impact factor: 5.157

2. Solubilization of glucocerebrosidase from human placenta and demonstration of a phospholipid requirement for its catalytic activity.

Authors: G L Dale; D G Villacorte; E Beutler
Journal: Biochem Biophys Res Commun Date: 1976-08-23 Impact factor: 3.575

3. Enzyme loading of erythrocytes.

Authors: G M Ihler; R H Glew; F W Schnure
Journal: Proc Natl Acad Sci U S A Date: 1973-09 Impact factor: 11.205

4. Affinity chromatography on immobilised adenosine 5'-monophosphate. 2. Some parameters relating to the selection and concentration of the immobilised ligand.

Authors: M J Harvey; C R Lowe; D B Craven; P D Dean
Journal: Eur J Biochem Date: 1974-01-16

5. A simplified method for cyanogen bromide activation of agarose for affinity chromatography.

Authors: S C March; I Parikh; P Cuatrecasas
Journal: Anal Biochem Date: 1974-07 Impact factor: 3.365

6. Adsorbents for affinity chromatography. Use of N-hydroxysuccinimide esters of agarose.

Authors: P Cuatrecasas; I Parikh
Journal: Biochemistry Date: 1972-06-06 Impact factor: 3.162

7. Beta-glucosidase activities in human tissues. Findings in Gaucher's disease.

Authors: B Hultberg; P A Ockerman
Journal: Clin Chim Acta Date: 1970-04 Impact factor: 3.786

8. Enzyme replacement therapy for the sphingolipidoses.

Authors: R O Brady; P G Pentchev; A E Gal; S R Hibbert; J M Quirk; G E Mook; J W Kusiak; J F Tallman; A S Dekaban
Journal: Adv Exp Med Biol Date: 1976 Impact factor: 2.622

9. Hydrophobic chromatography: use for purification of glycogen synthetase.

Authors: S Shaltiel; Z Er-El
Journal: Proc Natl Acad Sci U S A Date: 1973-03 Impact factor: 11.205

10. The diagnosis of the adult type of Gaucher's disease and its carrier state by demonstration of deficiency of beta-glucosidase activity in peripheral blood leukocytes.

Authors: E Beutler; W Kuhl
Journal: J Lab Clin Med Date: 1970-11

14 in total

1. Production of active human glucocerebrosidase in seeds of Arabidopsis thaliana complex-glycan-deficient (cgl) plants.

Authors: Xu He; Jason D Galpin; Michael B Tropak; Don Mahuran; Thomas Haselhorst; Mark von Itzstein; Daniel Kolarich; Nicolle H Packer; Yansong Miao; Liwen Jiang; Gregory A Grabowski; Lorne A Clarke; Allison R Kermode
Journal: Glycobiology Date: 2011-11-07 Impact factor: 4.313

Enzyme replacement therapy in Gaucher's disease: a rapid, high-yield method for purification of glucocerebrosidase.

1. A reinvestigation of the cerebroside deposited in Gaucher's disease.

2. Solubilization of glucocerebrosidase from human placenta and demonstration of a phospholipid requirement for its catalytic activity.

3. Enzyme loading of erythrocytes.

4. Affinity chromatography on immobilised adenosine 5'-monophosphate. 2. Some parameters relating to the selection and concentration of the immobilised ligand.

5. A simplified method for cyanogen bromide activation of agarose for affinity chromatography.

6. Adsorbents for affinity chromatography. Use of N-hydroxysuccinimide esters of agarose.

7. Beta-glucosidase activities in human tissues. Findings in Gaucher's disease.

8. Enzyme replacement therapy for the sphingolipidoses.

9. Hydrophobic chromatography: use for purification of glycogen synthetase.

10. The diagnosis of the adult type of Gaucher's disease and its carrier state by demonstration of deficiency of beta-glucosidase activity in peripheral blood leukocytes.

1. Production of active human glucocerebrosidase in seeds of Arabidopsis thaliana complex-glycan-deficient (cgl) plants.

2. Enzyme replacement therapy in Gaucher's disease: preliminary clinical trial of a new enzyme preparation.

Review 3. Binding interactions of glycoproteins with lectins.

4. Incorporation of glucocerebrosidase into Gaucher's disease monocytes in vitro.

Review 5. Gaucher disease as a paradigm of current issues regarding single gene mutations of humans.

6. Binding, internalization, and degradation of mannose-terminated glucocerebrosidase by macrophages.

7. Immunological evaluation of repeated administration of erythrocyte-entrapped protein to C3H/HeJ mice.

8. Gaucher disease: the effects of phosphatidylserine on glucocerebrosidase from normal and Gaucher fibroblasts.

9. Glucocerebrosidase "processing" and gene expression in various forms of Gaucher disease.

10. Failure of alglucerase infused into Gaucher disease patients to localize in marrow macrophages.