Literature DB >> 207338

Pilot scale purification of alpha-galactosidase A from Cohn fraction IV-1 of human plasma.

D F Bishop, D E Wampler, J T Sgouris, R J Bonefeld, D K Anderson, M C Hawley, C C Sweeley.   

Abstract

Human plasma alpha-galactosidase A (alpha-D-galactoside galactohydrolase, EC 3.2.1.22) was purified 7000-fold over plasma levels from Cohn Fraction IV-1. The yield per kg starting material averaged 11 000 units (nmol galactose liberated per h) and the specific activity was about 600 units per mg protein with 4-methylumbelliferyl-alpha-D-galactoside. The ratio of 4-methylumbelliferyl-alpha-galactosidase to ceramide trihexosidase activities was 6.2. Both activities were heat labile and exhibited the same relative mobilities on polyacrylamide gel electrophoresis. Enzymatic activity was stable for at least 4 months at 4 and -20 degrees C. The endotoxin concentration of this preparation averaged 0.26 mg per mg protein.

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Year:  1978        PMID： 207338     DOI： 10.1016/0005-2744(78)90109-2

Source DB:  PubMed          Journal:  Biochim Biophys Acta        ISSN： 0006-3002


  11 in total

1.  Enzyme therapy in Fabry disease: differential in vivo plasma clearance and metabolic effectiveness of plasma and splenic alpha-galactosidase A isozymes.

Authors:  R J Desnick; K J Dean; G Grabowski; D F Bishop; C C Sweeley
Journal:  Proc Natl Acad Sci U S A       Date:  1979-10       Impact factor: 11.205

2.  Enzymatic defect in "X-linked" sideroblastic anemia: molecular evidence for erythroid delta-aminolevulinate synthase deficiency.

Authors:  P D Cotter; M Baumann; D F Bishop
Journal:  Proc Natl Acad Sci U S A       Date:  1992-05-01       Impact factor: 11.205

3.  Inherited X-chromosome inverted tandem duplication in a male traced to a grandparental mitotic error.

Authors:  S Schwartz; M F Schwartz; S R Panny; C J Peterson; E Waters; M M Cohen
Journal:  Am J Hum Genet       Date:  1986-05       Impact factor: 11.025

4.  Human alpha-galactosidase A: glycosylation site 3 is essential for enzyme solubility.

Authors:  Y A Ioannou; K M Zeidner; M E Grace; R J Desnick
Journal:  Biochem J       Date:  1998-06-15       Impact factor: 3.857

5.  X-linked sideroblastic anemia due to carboxyl-terminal ALAS2 mutations that cause loss of binding to the β-subunit of succinyl-CoA synthetase (SUCLA2).

Authors:  David F Bishop; Vassili Tchaikovskii; A Victor Hoffbrand; Marie E Fraser; Steven Margolis
Journal:  J Biol Chem       Date:  2012-06-27       Impact factor: 5.157

6.  Molecular expression and characterization of erythroid-specific 5-aminolevulinate synthase gain-of-function mutations causing X-linked protoporphyria.

Authors:  David F Bishop; Vassili Tchaikovskii; Irina Nazarenko; Robert J Desnick
Journal:  Mol Med       Date:  2013-03-05       Impact factor: 6.354

7.  First-trimester prenatal diagnosis of Tay-Sachs disease.

Authors:  G A Grabowski; J R Kruse; J D Goldberg; K Chockkalingam; R E Gordon; K J Blakemore; M J Mahoney; R J Desnick
Journal:  Am J Hum Genet       Date:  1984-11       Impact factor: 11.025

8.  Fabry disease: isolation of a cDNA clone encoding human alpha-galactosidase A.

Authors:  D H Calhoun; D F Bishop; H S Bernstein; M Quinn; P Hantzopoulos; R J Desnick
Journal:  Proc Natl Acad Sci U S A       Date:  1985-11       Impact factor: 11.205

9.  Late-onset X-linked sideroblastic anemia. Missense mutations in the erythroid delta-aminolevulinate synthase (ALAS2) gene in two pyridoxine-responsive patients initially diagnosed with acquired refractory anemia and ringed sideroblasts.

Authors:  P D Cotter; A May; E J Fitzsimons; T Houston; B E Woodcock; A I al-Sabah; L Wong; D F Bishop
Journal:  J Clin Invest       Date:  1995-10       Impact factor: 14.808

Review 10.  Reflections on my career in analytical chemistry and biochemistry.

Authors:  Charles C Sweeley
Journal:  Proc Jpn Acad Ser B Phys Biol Sci       Date:  2010       Impact factor: 3.493
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