Literature DB >> 945735

Studies on human N-acetyl-Beta-d-hexosaminidase C separated from neonatal brain.

G T Besley, D M Broadhead.   

Abstract

Human brain hexosaminidase C was separated from isoenzymes A and B by Sephadex G-200 gel filtration. Properties of the enzyme were studied, particularly its isoelectric-focusing profile, pI4.80. These findings indicate that hexosaminidase C is identical with the major residual component of Sandhoff fibroblasts with respect to substrate specificity, pI and activity pH optimum.

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Year:  1976        PMID： 945735      PMCID： PMC1172820          DOI： 10.1042/bj1550205

Source DB:  PubMed          Journal:  Biochem J        ISSN： 0264-6021            Impact factor:   3.857


  19 in total

1.  Studies on hexosaminidase C in cultured skin fibroblasts from patients with Sandhoff's disease.

Authors:  D M Broadhead; G T Besley
Journal:  Biochem Soc Trans       Date:  1975       Impact factor: 5.407

2.  Characterization of Hex S, the major residual beta hexosaminidase activity in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease).

Authors:  J U Ikonne; M C Rattazzi; R J Desnick
Journal:  Am J Hum Genet       Date:  1975-09       Impact factor: 11.025

3.  N-acetyl-beta-D-hexosaminidase component A. Different forms in human tissues and fluids.

Authors:  J U Ikonne; R B Ellis
Journal:  Biochem J       Date:  1973-11       Impact factor: 3.857

4.  Tay-Sachs and Sandhoff's disease: intergenic complementation after somatic cell hybridization.

Authors:  H Galjaard; A Hoogeveen; H A de Wit-Verbeek; A J Reuser; W Keijzer; A Westerveld; D Bootsma
Journal:  Exp Cell Res       Date:  1974-08       Impact factor: 3.905

5.  Separation and properties of human brain hexosaminidase C.

Authors:  I Braidman; M Carroll; N Dance; D Robinson
Journal:  Biochem J       Date:  1974-11       Impact factor: 3.857

6.  Differences between the N-acetyl hexosaminidase isozymes in serum and tissues.

Authors:  D M Swallow; D C Stokes; G Corney; H Harris
Journal:  Ann Hum Genet       Date:  1974-01       Impact factor: 1.670

7.  Electrophoretic study of hexosaminidases. Hexosaminidase C.

Authors:  L Poenaru; J C Dreyfus
Journal:  Clin Chim Acta       Date:  1973-02-12       Impact factor: 3.786

8.  Cystic fibrosis and liver -galactosidase and -glucosidase.

Authors:  J Butterworth; A D Bain; W M McCrae
Journal:  Clin Chim Acta       Date:  1972-10       Impact factor: 3.786

9.  On the molecular basis of Sandhoff's disease.

Authors:  H H Ropers; U Schwantes
Journal:  Humangenetik       Date:  1973

10.  Hexosaminidase C in Tay-Sachs and Sandhoff disease.

Authors:  E Penton; L Poenaru; J C Dreyfus
Journal:  Biochim Biophys Acta       Date:  1975-05-23
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  6 in total

1.  Mammalian cells contain a second nucleocytoplasmic hexosaminidase.

Authors:  Martin Gutternigg; Dubravko Rendić; Regina Voglauer; Thomas Iskratsch; Iain B H Wilson
Journal:  Biochem J       Date:  2009-04-01       Impact factor: 3.857

Review 2.  Glycosphingolipid hydrolases: properties and molecular genetics.

Authors:  M Wan Ho; A G Norden; J A Alhadeff; J S O'Brien
Journal:  Mol Cell Biochem       Date:  1977-10-07       Impact factor: 3.396

3.  Prenatal diagnosis of Tay-Sachs disease. Reflectometry of hexosaminidase A, B, and C/S bands on zymograms.

Authors:  B Kustermann-Kuhn; K Harzer
Journal:  Hum Genet       Date:  1983       Impact factor: 4.132

4.  The multiple forms and kinetic properties of the N-acetyl-beta-D-hexosaminidases from colonic tumours and mucosa of rats treated with 1,2-dimethylhydrazine.

Authors:  N Mian; D G Herries; D M Cowen; E A Batte
Journal:  Biochem J       Date:  1979-01-01       Impact factor: 3.857

5.  Enzyme activities and phospholipid storage patterns in brain and spleen samples from Niemann-Pick disease variants: a comparison of neuropathic and non-neuropathic forms.

Authors:  G T Besley; M Elleder
Journal:  J Inherit Metab Dis       Date:  1986       Impact factor: 4.982

6.  The use of natural and artifical substrates in the prenatal diagnosis of Krabbe's disease.

Authors:  G T Besley
Journal:  J Inherit Metab Dis       Date:  1978       Impact factor: 4.982
  6 in total

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