Biochemical studies on leukocyte and fibroblast human β-galactosidase

Clinical Biochemistry

Volumn 32, Issue 3, 1999, Pages 167-170

  Coelho, J C ^a,b   Sopelsa, A M I ^a,b   Tobo, P R ^a,b   Severini, M H A ^b   Silva, C D ^a,b   Giugliani, R ^a,b  

^a FEDERAL UNIVERSITY OF RIO GRANDE DO SUL   (Brazil)

^b Clinical Hospital of Porto Alegre   (Brazil)

Author keywords

Kinetics; pH optimum; Thermostability; Galactosidase

Indexed keywords

BETA GALACTOSIDASE;

ARTICLE; ENZYME ACTIVITY; ENZYME ANALYSIS; ENZYME ASSAY; ENZYME INACTIVATION; FIBROBLAST; GLOBOID CELL LEUKODYSTROPHY; GM1 GANGLIOSIDOSIS; INBORN ERROR OF METABOLISM; LEUKOCYTE; PH; PRIORITY JOURNAL; THERMOSTABILITY;

EID: 0033012595     PISSN: 00099120     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0009-9120(98)00107-6     Document Type: Article

References (18)

1. Okada S., O'Brien J.S. Generalized gangliosidosis β-galactosidase deficiency . Science. 160:1968;1002-1004.
2. Arbisser A.I., Donnelly K.A., Scott C.I., et al. Mild Morquion Syndrome (MPS IVB) with keratan sulfaturia but normal 6-sulfatase activity. Am J Med Genet. 1:1977;195-197.
3. Wenger D.A., Tarby T.J., Wharton C. Macular cherry-red spots and myoclonus with dementia Coexistent neuraminidase and β-galactosidase deficiencies . Biochem Biophys Res Commun. 82:1978;589-593.
4. Singer H.S., Schafer I.A. White cell β-galactosidase activity. N Engl J Med. 282:1970;571-578.
5. O'Brien J.S. The gangliosidosis. Stanbury J.B., Wingaarden J.B., Fredrickson D.S. The metabolic basis of inherited disease. 1978;841-862 McGraw-Hill, New York.
6. Suzuki Y., Sakuraba H., Oshima A. β-galactosidase deficiency (β-galactosidosis): GM1 gangliosidosis and Morquio B disease. Scriver C.R., Beaudet A.L., Sly W.S., Valle D. The metabolic and molecular basis of inherited disease. 1995;2785-2823 McGraw-Hill, New York.
7. Benson P.F., Fenson A.H. The sphingolipidosis and oher lipid stored disorders. Benson P.F., Fenson A.H. Genetic biochemical disorders. 1985;89-94 Oxford University Press, New York.
8. Pinski L., Powell E., Callahan J. GM1 gangliosidosis types 1 and 2 enzymatic differences in cultured fibroblasts . Nature. 228:1970;1093-1095.
9. Mutoh T., Naoi M., Takahashi A., Hoshino M., Nagai Y., Nagatsu T. Atypical adult GM1 gangliosid. Neurology. 36:1986;1237-1241.
10. Furuya T., Suzuki Y., Momoi T. Acid β-galactosidase from human fibroblasts. A microscale purification method monitored by a highly sensitive enzyme assay. J Biochem. 99:1986;437-443.
11. Mutoh T., Naoi M., Toshiharu N., et al. Purification and characterization of human liver β-galactosidase from a patient with the adult form of GM1 gangliosidosis and a normal control. Biochim Biophys Acta. 964:1988;244-253.
12. Skoog W.A., Beck W.S. Studies on the fibrinogen, dextran and phytohemaglutinin methods of isolating leukocytes. Blood. 11:1956;436-454.
13. Suzuki K. Globoid cell leukodystrophy (Krabbe disease) and GM1 gangliosidosis. Glew R.H., Peters S.P. Practical enzimology of sphingolipidosis. 1977;101-136 Alan R. Liss, New York.
14. Lowry O.H., Rosebrough N.J., Farr A.L., Randall R.J. Protein measurement with the folin phenol reagent. J Biol Chem. 193:1951;265-275.
15. Lineweaver H., Burk D. The determination of enzyme dissociation constants. J Am Chem Soc. 56:1934;658-666.
16. Farrel D.F., MacMartin M.P. GM1 gangliosidosis enzymatic variation in a single family . Ann Neurol. 9:1981;232-236.
17. Kolodny E.H., Mumford R.A. Human leukocyte acid hydrolases: characterization of eleven lysosomal enzymes and study of reaction conditions for their automated analysis. Clin Chim Acta. 70:1976;247-257.
18. Ho M.W., O'Brien J.S. Stimulation of acid β-galactosidase activity by chloride ions. Clin Chim Acta. 32:1971;531-534.