Immunological Properties of N-acetyl-beta-D-glucosaminidase of Normal Human Liver and of GM2-gangliosidosis Liver
Overview
Authors
Affiliations
Antisera were raised to a partially purified preparation of human liver hexosaminidase and to highly purified preparations of hexosaminidase isoenzymes A and B. All the antisera precipitated the enzyme in an enzymically active form, which could be located on immunodiffusion and immunoelectrophoretic gels by using a histochemical substrate. The antisera to the purified isoenzymes were shown to react with hexosaminidase from human liver, kidney, brain and spleen, but did not cross-react with human liver beta-glucosidase, beta-galactosidase, alpha-mannosidase, beta-xylosidase, arylsulphatase or acid phosphatase. Hexosaminidases A and B were immunologically identical. The immunological properties of the hexosaminidases from livers of patients with three types of GM(2)-gangliosidoses were closely similar. No evidence could be found for cross-reacting material in enzyme-deficient states.
Skovby F, Kraus J, Redlich C, ROSENBERG L Am J Hum Genet. 1982; 34(1):73-83.
PMID: 7081217 PMC: 1685209.
Ganglioside storage diseases: an updated review.
OBrien J Ital J Neurol Sci. 1981; 2(3):219-26.
PMID: 6804413 DOI: 10.1007/BF02335401.
Glossl J, Lembeck K, Gamse G, Kresse H Hum Genet. 1980; 54(1):87-91.
PMID: 6156114 DOI: 10.1007/BF00279054.
Lalley P, Rattazzi M, Shows T Proc Natl Acad Sci U S A. 1974; 71(4):1569-73.
PMID: 4524661 PMC: 388272. DOI: 10.1073/pnas.71.4.1569.
Separation and properties of human brain hexosaminidase C.
Braidman I, Carroll M, DANCE N, Robinson D Biochem J. 1974; 143(2):295-301.
PMID: 4477952 PMC: 1168384. DOI: 10.1042/bj1430295.