Literature DB >> 7396862

Comparison of the alpha-mannosidases in fibroblast cultures from patients with mannosidosis and mucolipidosis II and from controls.

D J Halley, B G Winchester, L J Burditt, A d'Azzo, D Robinson, H Galjaard.   

Abstract

The intracellular and extracellular acidic alpha-mannosidase in cultures of fibroblasts from mucolipidosis-II patients has normal kinetics. The extracellular activity in cultures of cells from mannosidosis patients is normal, but a mutant enzyme is associated with the cell surface and intracellular fraction. The results support the involvement of membrane cycling and recognition markers in lysosomal enzyme localization.

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Year:  1980        PMID: 7396862      PMCID: PMC1161824          DOI: 10.1042/bj1870541

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


  15 in total

1.  The relationship between different forms of human alpha-mannosidase.

Authors:  M A Chester; A Lundblad; P K Masson
Journal:  Biochim Biophys Acta       Date:  1975-06-24

2.  Residual altered alpha-mannosidase in human mannosidosis.

Authors:  A L Beaudet; B L Nichols
Journal:  Biochem Biophys Res Commun       Date:  1976-01-12       Impact factor: 3.575

3.  A recognition marker required for uptake of a lysosomal enzyme by cultured fibroblasts.

Authors:  S Hickman; L J Shapiro; E F Neufeld
Journal:  Biochem Biophys Res Commun       Date:  1974-03-15       Impact factor: 3.575

4.  A hypothesis for I-cell disease: defective hydrolases that do not enter lysosomes.

Authors:  S Hickman; E F Neufeld
Journal:  Biochem Biophys Res Commun       Date:  1972-11-15       Impact factor: 3.575

5.  Multiple lysosomal enzyme deficiency due to enzyme leakage?

Authors:  U N Wiesmann; J Lightbody; F Vassella; N N Herschkowitz
Journal:  N Engl J Med       Date:  1971-01-14       Impact factor: 91.245

6.  Human mannosidosis--the enzyme defect.

Authors:  M Carroll; N Dance; P K Masson; D Robinson; B G Winchester
Journal:  Biochem Biophys Res Commun       Date:  1972-10-17       Impact factor: 3.575

7.  Activation by ferrous ions of an alpha-mannosidase in rat testis and liver.

Authors:  S M Snaith
Journal:  Biochem Soc Trans       Date:  1973-11       Impact factor: 5.407

8.  Evidence for a non-lysosomal alpha-mannosidase in rat liver homogenates.

Authors:  C A Marsh; G C Gourlay
Journal:  Biochim Biophys Acta       Date:  1971-04-14

9.  [Alpha-mannosidase activity of different human biological fluids].

Authors:  J M Chéron; P Rahimulla; J E Courtois; R Truhaut
Journal:  C R Acad Hebd Seances Acad Sci D       Date:  1975-05-26

10.  Mannosidosis in Angus cattle. The enzymic defect.

Authors:  N C Phillips; D Robinson; B G Winchester; R D Jolly
Journal:  Biochem J       Date:  1974-02       Impact factor: 3.857

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  1 in total

1.  Apparently normal extracellular acidic alpha-mannosidase in fibroblast cultures from patients with mannosidosis.

Authors:  Y Ben-Yoseph; C L DeFranco; J Charrow; L C Hahn; H L Nadler
Journal:  Am J Hum Genet       Date:  1982-01       Impact factor: 11.025

  1 in total

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