Literature DB >> 2970846

Identification of beta-N-acetylhexosaminidase A in mouse tissues with the fluorigenic substrate 4-methylumbelliferyl-beta-N-acetylglucosamine 6-sulphate.

T Beccari1, A Orlacchio, J L Stirling.   

Abstract

beta-N-Acetylhexosaminidase from mouse tissue was separated into its constituent isoenzymes on DEAE-cellulose and its activity was monitored with 4-methylumbelliferyl-beta-N-acetylglucosamine and 4-methylumbelliferyl-beta-N-acetylglucosamine 6-sulphate. Forms corresponding to the human isoenzymes A (acidic), B (basic) and an 'intermediate' form were present in mouse liver and spleen, whereas in kidney the B and 'intermediate' forms predominated, with A present only as a minor component. In brain the 'intermediate', A and C activities were detected. Testis had predominantly A activity, whereas epididymis, the tissue with the highest specific activity of beta-N-acetylhexosaminidase, had an abundance of the 'intermediate' form, but was almost entirely lacking in the A form.

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Year:  1988        PMID： 2970846      PMCID： PMC1149187          DOI： 10.1042/bj2520617

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


  15 in total

1.  Diagnosis and carrier detection of Tay-Sachs disease: direct determination of hexosaminidase A using 4-methylumbelliferyl derivatives of beta-N-acetylglucosamine-6-sulfate and beta-N-acetylgalactosamine-6-sulfate.

Authors:  Y Ben-Yoseph; J E Reid; B Shapiro; H L Nadler
Journal:  Am J Hum Genet       Date:  1985-07       Impact factor: 11.025

2.  Intermediate forms of human beta-N-acetylhexosaminidase lack activity towards 4-methylumbelliferyl beta-N-acetylglucosaminide 6-sulphate.

Authors:  T Beccari; C Emiliani; R Hosseini; A Orlacchio; J L Stirling
Journal:  Biochem J       Date:  1987-06-15       Impact factor: 3.857

3.  Genetic control of levels of murine kidney glucuronidase mRNA in response to androgen.

Authors:  R Palmer; P M Gallagher; W L Boyko; R E Ganschow
Journal:  Proc Natl Acad Sci U S A       Date:  1983-12       Impact factor: 11.205

4.  Separation and properties of beta-galactosidase, beta-glucosidase, beta-glucuronidase and N-acetyl-beta-glucosaminidase from rat kidney.

Authors:  D Robinson; R G Price; N Dance
Journal:  Biochem J       Date:  1967-02       Impact factor: 3.857

5.  Isolation of cDNA clones coding for the alpha-subunit of human beta-hexosaminidase. Extensive homology between the alpha- and beta-subunits and studies on Tay-Sachs disease.

Authors:  R G Korneluk; D J Mahuran; K Neote; M H Klavins; B F O'Dowd; M Tropak; H F Willard; M J Anderson; J A Lowden; R A Gravel
Journal:  J Biol Chem       Date:  1986-06-25       Impact factor: 5.157

6.  Hormonal regulation of isoenzymes of N-acetyl-beta-glucosaminidase and beta-galactosidase during spermatogenesis in the rat.

Authors:  G C Majumder; S Lessin; R W Turkington
Journal:  Endocrinology       Date:  1975-04       Impact factor: 4.736

7.  Affinity purification and subunit structures of beta-N-acetylhexosaminidases A and B from boar epididymis.

Authors:  H C Parkes; J L Stirling; P Calvo
Journal:  Biochem J       Date:  1984-05-01       Impact factor: 3.857

8.  Usefulness of 4-methylumbelliferyl-6-sulfo-2-acetamido-2-deoxy-beta-D-glucopyrano sid e for the diagnosis of GM2 gangliosidoses in leukocytes.

Authors:  K Inui; D A Wenger
Journal:  Clin Genet       Date:  1984-10       Impact factor: 4.438

9.  Human beta-hexosaminidase alpha chain: coding sequence and homology with the beta chain.

Authors:  R Myerowitz; R Piekarz; E F Neufeld; T B Shows; K Suzuki
Journal:  Proc Natl Acad Sci U S A       Date:  1985-12       Impact factor: 11.205

10.  Evidence for two different active sites on human beta-hexosaminidase A. Interaction of GM2 activator protein with beta-hexosaminidase A.

Authors:  H J Kytzia; K Sandhoff
Journal:  J Biol Chem       Date:  1985-06-25       Impact factor: 5.157
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  5 in total

1.  Cloning and sequence analysis of a cDNA encoding the alpha-subunit of mouse beta-N-acetylhexosaminidase and comparison with the human enzyme.

Authors:  T Beccari; J Hoade; A Orlacchio; J L Stirling
Journal:  Biochem J       Date:  1992-07-15       Impact factor: 3.857

2.  Analysis of the patterns of expression of mRNAs for the alpha- and beta-subunits of the lysosomal enzyme beta-N-acetylhexosaminidase in mouse epididymis and testis.

Authors:  J L Stirling; T Beccari; J Hoade; F Pezzetti; M Calvitti; E Becchetti; A Orlacchio
Journal:  Histochem J       Date:  1991-10

3.  Disruption of murine Hexa gene leads to enzymatic deficiency and to neuronal lysosomal storage, similar to that observed in Tay-Sachs disease.

Authors:  M Cohen-Tannoudji; P Marchand; S Akli; S A Sheardown; J P Puech; C Kress; P Gressens; M C Nassogne; T Beccari; A L Muggleton-Harris
Journal:  Mamm Genome       Date:  1995-12       Impact factor: 2.957

4.  Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease.

Authors:  S Yamanaka; M D Johnson; A Grinberg; H Westphal; J N Crawley; M Taniike; K Suzuki; R L Proia
Journal:  Proc Natl Acad Sci U S A       Date:  1994-10-11       Impact factor: 11.205

Review 5.  Lysosomal Ceramide Metabolism Disorders: Implications in Parkinson's Disease.

Authors:  Silvia Paciotti; Elisabetta Albi; Lucilla Parnetti; Tommaso Beccari
Journal:  J Clin Med       Date:  2020-02-21       Impact factor: 4.241
  5 in total

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