Literature DB >> 7495557

In situ assessment of beta-hexosaminidase activity.

H D Lacorazza1, M Jendoubi.   

Abstract

We have adapted two methods to evaluate the beta-hexosaminidase (HEX) enzymatic activity in cultured cells, based on the use of (i) the fluorogenic substrate 4-methylumbelliferyl-6-sulfo-2-acetamido-2- deoxy-beta-D-glucopyranoside (MU-GlcNAc-6-SO4) and (ii) the naphthol AS-BI-N-acetyl-beta-D-glucosaminide and hexazotized pararosaniline. We demonstrate that both methods could be used for the HEX isoenzymes by comparing wild-type and mutant human fibroblast cell lines, deficient for either an alpha or beta subunit from Tay-Sachs and Sandhoff patients. This in situ cytochemical assessment of HEX activity offers a rapid evaluation to study the expression of this enzyme in a heterogeneous cell population such as in gene transfer experiments.

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Year:  1995        PMID： 7495557

Source DB:  PubMed          Journal:  Biotechniques        ISSN： 0736-6205            Impact factor:   1.993


  8 in total

1.  Effective gene therapy in an authentic model of Tay-Sachs-related diseases.

Authors:  M Begoña Cachón-González; Susan Z Wang; Andrew Lynch; Robin Ziegler; Seng H Cheng; Timothy M Cox
Journal:  Proc Natl Acad Sci U S A       Date:  2006-06-26       Impact factor: 11.205

2.  Gene transfer corrects acute GM2 gangliosidosis--potential therapeutic contribution of perivascular enzyme flow.

Authors:  M Begoña Cachón-González; Susan Z Wang; Rosamund McNair; Josephine Bradley; David Lunn; Robin Ziegler; Seng H Cheng; Timothy M Cox
Journal:  Mol Ther       Date:  2012-03-27       Impact factor: 11.454

3.  Widespread correction of central nervous system disease after intracranial gene therapy in a feline model of Sandhoff disease.

Authors:  V J McCurdy; H E Rockwell; J R Arthur; A M Bradbury; A K Johnson; A N Randle; B L Brunson; M Hwang; H L Gray-Edwards; N E Morrison; J A Johnson; H J Baker; N R Cox; T N Seyfried; M Sena-Esteves; D R Martin
Journal:  Gene Ther       Date:  2014-12-04       Impact factor: 5.250

4.  Therapeutic response in feline sandhoff disease despite immunity to intracranial gene therapy.

Authors:  Allison M Bradbury; J Nicholas Cochran; Victoria J McCurdy; Aime K Johnson; Brandon L Brunson; Heather Gray-Edwards; Stanley G Leroy; Misako Hwang; Ashley N Randle; Laura S Jackson; Nancy E Morrison; Rena C Baek; Thomas N Seyfried; Seng H Cheng; Nancy R Cox; Henry J Baker; M Begona Cachón-González; Timothy M Cox; Miguel Sena-Esteves; Douglas R Martin
Journal:  Mol Ther       Date:  2013-05-21       Impact factor: 11.454

5.  Reversibility of neuropathology in Tay-Sachs-related diseases.

Authors:  María-Begoña Cachón-González; Susan Z Wang; Robin Ziegler; Seng H Cheng; Timothy M Cox
Journal:  Hum Mol Genet       Date:  2013-09-20       Impact factor: 6.150

6.  Characterization of inducible models of Tay-Sachs and related disease.

Authors:  Timothy J Sargeant; Deborah J Drage; Susan Wang; Apostolos A Apostolakis; Timothy M Cox; M Begoña Cachón-González
Journal:  PLoS Genet       Date:  2012-09-20       Impact factor: 5.917

7.  AAV-mediated gene delivery in a feline model of Sandhoff disease corrects lysosomal storage in the central nervous system.

Authors:  Hannah E Rockwell; Victoria J McCurdy; Samuel C Eaton; Diane U Wilson; Aime K Johnson; Ashley N Randle; Allison M Bradbury; Heather L Gray-Edwards; Henry J Baker; Judith A Hudson; Nancy R Cox; Miguel Sena-Esteves; Thomas N Seyfried; Douglas R Martin
Journal:  ASN Neuro       Date:  2015-04-13       Impact factor: 4.146

8.  Therapeutic benefit after intracranial gene therapy delivered during the symptomatic stage in a feline model of Sandhoff disease.

Authors:  Victoria J McCurdy; Aime K Johnson; Heather L Gray-Edwards; Ashley N Randle; Allison M Bradbury; Nancy E Morrison; Misako Hwang; Henry J Baker; Nancy R Cox; Miguel Sena-Esteves; Douglas R Martin
Journal:  Gene Ther       Date:  2020-09-03       Impact factor: 5.250
  8 in total

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