DOI: 10.1111/j.1471-4159.1983.tb12667.x. The Protein Activator Specific for the Enzymic Hydrolysis of GM<sub>2</sub> Ganglioside in Normal Human Brain and Brains of Three Types of GM<sub>2</sub> Gangliosidosis

The Protein Activator Specific for the Enzymic Hydrolysis of GM2 Ganglioside in Normal Human Brain and Brains of Three Types of GM2 Gangliosidosis

10.1111/j.1471-4159.1983.tb12667.x

Crossref journal-article

Wiley

Journal of Neurochemistry (311)

Abstract

Abstract: In order to understand the etiology of Type AB GM2 gangliosidosis, we have purified and characterized the activator protein (GM2 activator) specific for the enzymic hydrolysis of GM2 ganglioside from normal human brain. The purified activator from human brain moved as one major protein band in various electrophoretic systems. We have also prepared the antiserum against this activator. The levels and the nature of GM2 activator and β‐hexosaminidase A were examined in the brains of five cases of GM2. gangliosidosis—one Type B, two Type O, and two Type AB. We found that the levels of GM2 activator in both Type B and Type O cases were markedly elevated, and that the two Type AB cases were the results of different causes. One case had a defective β hexosaminidase A and an elevated level of GM2 activator. Although this defective β‐hexosaminidase A could hydrolyze synthetic substrates, it was inactive in the cleavage of natural glycosphingolipids in the presence of the GM2 activator. It could, however, hydrolyze asialo‐GM2 and GbOse4Cer in the presence of sodium taurodeoxycholate. The other case had normal β‐hexosaminidase A, but had a very low level of GM2 activator when analyzed by in vitro assay, suggesting the deficiency of this activator. By immunoelectrophoresis, this case was found to be completely devoid of the protein that cross‐reacts with the antiserum against the GM2 activator.

Bibliography

Hirabayashi, Y., Li, Y., & Li, S. (1983). The Protein Activator Specific for the Enzymic Hydrolysis of GM2 Ganglioside in Normal Human Brain and Brains of Three Types of GM2 Gangliosidosis. Journal of Neurochemistry, 40(1), 168â175. Portico.

Authors 3

Yoshio Hirabayashi (first)
Yu‐Teh Li (additional)
Su‐Chen Li (additional)

References 25 Referenced 59

10.1073/pnas.75.8.3979
10.1515/bchm2.1979.360.2.1837
10.1111/j.1749-6632.1964.tb14213.x
10.1016/S0021-9258(19)86663-2 / J. Biol. Chem. / Studies on human liver α‐galactosidases. 1. Purification of α‐galactosidase A and its enzymatic properties with glycolipid and oligosaccharide substrates by Dean K. J. (1979)
10.1016/0076-6879(72)28010-7
10.1007/BF00705807
10.1016/0006-3002(53)90233-9 / Biochim. Biophys. Acta / Methode permettant I'étude conjugée des proprigtes électrophorétiques et immunochimiques d'un mélange de protéines. Application au sérum sanguin by Grabar P. (1953)
10.3181/00379727-142-37047
10.1203/00006450-198203000-00011
10.1016/0014-5793(72)80680-X
10.1016/0006-291X(75)90309-5
10.1016/S0021-9258(17)33814-0
10.1016/S0021-9258(19)86559-6 / J. Biol. Chem. / Evidence for the presence of two separate protein activators for the enzymic hydrolysis of GM1 and GM2 gangliosides by Li S.‐C. (1979)
10.1016/0006-291X(81)91285-7
10.1016/S0021-9258(19)69152-0 / J. Biol. Chem. / A protein activator for the enzymic hydrolysis of GM2 ganglioside by Li S.‐C. (1981)
10.1016/S0021-9258(19)52451-6
{'key': 'e_1_2_2_18_1', 'first-page': '841', 'volume-title': 'The Metabolic Basis of Inherited Disease', 'author': "O'Brien J. S.", 'year': '1978'} / The Metabolic Basis of Inherited Disease by O'Brien J. S. (1978)
10.1111/j.1399-0004.1977.tb01296.x
10.1016/0076-6879(72)28034-X
{'key': 'e_1_2_2_21_1', 'first-page': '2054', 'article-title': 'Studies on human β‐d‐N‐acetylhexosaminidases. III. Biochemical genetics of Tay‐Sachs and Sandhoffs disease', 'volume': '249', 'author': 'Srivastava S.', 'year': '1974', 'journal-title': 'J. Biol. Chem.'} / J. Biol. Chem. / Studies on human β‐d‐N‐acetylhexosaminidases. III. Biochemical genetics of Tay‐Sachs and Sandhoffs disease by Srivastava S. (1974)
{'key': 'e_1_2_2_22_1', 'first-page': '464', 'article-title': 'Gangliosides, isolation', 'volume': '6', 'author': 'Svennerholm L.', 'year': '1972', 'journal-title': 'Methods Curbohydr. Chem.'} / Methods Curbohydr. Chem. / Gangliosides, isolation by Svennerholm L. (1972)
10.1016/S0021-9258(19)44435-9
10.1016/S0021-9258(19)63330-2 / J. Biol. Chem. / α‐N‐Acetylgalactosaminidase from the limpet, Patella vulguta by Uda Y. (1977)
10.1016/S0021-9258(18)94333-4
10.1111/j.1471-4159.1980.tb12515.x

Dates

Type	When
Created	18 years, 8 months ago (Dec. 13, 2006, 7:37 a.m.)
Deposited	1 year, 10 months ago (Oct. 19, 2023, 12:26 p.m.)
Indexed	11 months, 2 weeks ago (Sept. 7, 2024, 5:15 a.m.)
Issued	42 years, 7 months ago (Jan. 1, 1983)
Published	42 years, 7 months ago (Jan. 1, 1983)
Published Online	18 years, 8 months ago (Dec. 12, 2006)
Published Print	42 years, 7 months ago (Jan. 1, 1983)

Funders 0

None

BibTeX

@article{Hirabayashi_1983, title={The Protein Activator Specific for the Enzymic Hydrolysis of GM2 Ganglioside in Normal Human Brain and Brains of Three Types of GM2 Gangliosidosis}, volume={40}, ISSN={1471-4159}, url={http://dx.doi.org/10.1111/j.1471-4159.1983.tb12667.x}, DOI={10.1111/j.1471-4159.1983.tb12667.x}, number={1}, journal={Journal of Neurochemistry}, publisher={Wiley}, author={Hirabayashi, Yoshio and Li, Yu‐Teh and Li, Su‐Chen}, year={1983}, month=jan, pages={168–175} }

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