Literature DB >> 6881139

Presence of activator proteins for the enzymic hydrolysis of GM1 and GM2 gangliosides in normal human urine.

Y T Li, I A Muhiudeen, R DeGasperi, Y Hirabayashi, S C Li.   

Abstract

Normal human urine has been found to contain activator proteins that stimulate the enzymic hydrolysis of GM1 and GM2 gangliosides. These two activators were partially purified by Sephadex G-200 filtration and DEAE-Sephadex A-50 chromatography. The presence of these two activators was assayed by demonstrating the stimulation of the in vitro hydrolysis of GM1 and GM2 gangliosides. As little as 50 ml of urine is sufficient to detect the presence of these two activators. The crude activator preparation from normal urine was also found to stimulate the hydrolysis of galactosylceramide sulfate by arylsulfatase A.

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Year:  1983        PMID: 6881139      PMCID: PMC1685737     

Source DB:  PubMed          Journal:  Am J Hum Genet        ISSN: 0002-9297            Impact factor:   11.025


  16 in total

1.  The activator of cerebroside sulphatase. Purification from human liver and identification as a protein.

Authors:  G Fischer; H Jatzkewitz
Journal:  Hoppe Seylers Z Physiol Chem       Date:  1975-05

2.  Gaucher's disease: deficiency of 'acid' -glucosidase and reconstitution of enzyme activity in vitro.

Authors:  M W Ho; J S O'Brien
Journal:  Proc Natl Acad Sci U S A       Date:  1971-11       Impact factor: 11.205

3.  beta-Glucosidase activator protein from bovine spleen ("coglucosidase").

Authors:  B L Berent; N S Radin
Journal:  Arch Biochem Biophys       Date:  1981-04-15       Impact factor: 4.013

4.  A protein activator for the enzymic hydrolysis of GM2 ganglioside.

Authors:  S C Li; Y Hirabayashi; Y T Li
Journal:  J Biol Chem       Date:  1981-06-25       Impact factor: 5.157

5.  AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2.

Authors:  E Conzelmann; K Sandhoff
Journal:  Proc Natl Acad Sci U S A       Date:  1978-08       Impact factor: 11.205

6.  Purification and properties of a heat-stable glucocerebrosidase activating factor from control and Gaucher spleen.

Authors:  S P Peters; P Coyle; C J Coffee; R H Glew
Journal:  J Biol Chem       Date:  1977-01-25       Impact factor: 5.157

7.  An activator stimulating the enzymic hydrolysis of sphingoglycolipids.

Authors:  S C Li; Y T Li
Journal:  J Biol Chem       Date:  1976-02-25       Impact factor: 5.157

8.  Niemann-Pick disease, Type C: evidence for the deficiency of an activating factor stimulating sphingomyelin and glucocerebroside degradation.

Authors:  H Christomanou
Journal:  Hoppe Seylers Z Physiol Chem       Date:  1980-10

9.  Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A.

Authors:  E Conzelmann; K Sandhoff
Journal:  Hoppe Seylers Z Physiol Chem       Date:  1979-12

10.  Purification and properties of the hexosaminidase A-activating protein from human liver.

Authors:  P Hechtman; D LeBlanc
Journal:  Biochem J       Date:  1977-12-01       Impact factor: 3.857

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  3 in total

1.  Animal models of human cerebellar ataxias: a cornerstone for the therapies of the twenty-first century.

Authors:  Mario Manto; Daniele Marmolino
Journal:  Cerebellum       Date:  2009-09       Impact factor: 3.847

2.  Canine GM1-gangliosidosis. A clinical, morphologic, histochemical, and biochemical comparison of two different models.

Authors:  J Alroy; U Orgad; R DeGasperi; R Richard; C D Warren; K Knowles; J G Thalhammer; S S Raghavan
Journal:  Am J Pathol       Date:  1992-03       Impact factor: 4.307

3.  Genetic complementation in somatic cell hybrids of cerebroside sulfatase activator deficiency and metachromatic leukodystrophy fibroblasts.

Authors:  H Kihara; K K Tsay; A L Fluharty
Journal:  Hum Genet       Date:  1984       Impact factor: 4.132

  3 in total

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