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1. A method is described for the rapid isolation of alpha-galactosidases A and B (alpha-D-galactoside galactohydrolase, EC 3.2.1.22) from normal human liver. 2. When the same method is applied to Fabry liver, most of the alpha-galactosidase activity is recovered in the fraction corresponding to normal alpha-galactosidase B. In agreement with Romeo, G., D'Urso, M., Pisacane, A., Blum, E., De Falco, A. and Ruffilli, A. (1975) Biochem. Genet. 13, 615-628) [18], a small amount of alpha-galactosidase activity is found in the fraction corresponding to normal alpha-galactosidase A. 3. The kinetic properties of the B-like activity from Fabry liver are similar to those of normal alpha-galactosidase B. In agreement with Romeo et al. [18], it was found that the kinetic properties of the A-like activity from Fabry liver are similar to those of normal alpha-galactosidase A. 4. Using antisera raised against normal alpha-galactosidase A and normal alpha-galactosidase B, it is shown that the normal alpha-galactosidase isoenzymes are immunologically distinct and that the B-like activity from Fabry liver is immunologically related to normal alpha-galactosidase B. Furthermore, the A-like activity from Fabry liver is immunologically related to normal alpha-galactosidase B and not to normal alpha-galactosidase A. 5. Normal alpha-galactosidase B is converted into an A-like form during storage. 6. It is concluded that the B-like alpha-galactosidase in Fabry tissues is identical to normal alpha-galactosidase B, and that the small amount of A-like activity found in Fabry material is due to a modified form of alpha-galactosidase B.
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
May 1977,
Human genetics,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
October 1979,
European journal of biochemistry,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
January 1975,
Biochemical Society transactions,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
March 2007,
Journal of the American Society of Nephrology : JASN,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
January 1978,
Advances in experimental medicine and biology,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
October 1993,
Comparative biochemistry and physiology. B, Comparative biochemistry,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
January 1998,
Ryoikibetsu shokogun shirizu,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
December 1995,
Nihon rinsho. Japanese journal of clinical medicine,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
May 2015,
Clinica chimica acta; international journal of clinical chemistry,
A W Schram, and
M N Hamers, and
B Brouwer-Kelder, and
W E Donker-Koopman, and
J M Tager
November 1974,
American journal of human genetics,
