Leukocyte beta-glucosidase in homozygotes and heterozygotes for Gaucher disease.

@article{Raghavan1980LeukocyteBI,
  title={Leukocyte beta-glucosidase in homozygotes and heterozygotes for Gaucher disease.},
  author={Srinivasa S. Raghavan and Joanne Topol and Edwin H. Kolodny},
  journal={American journal of human genetics},
  year={1980},
  volume={32 2},
  pages={158-73}
}
Human leukocytes contain at least two isozymes of 4-methylumbelliferyl-beta-glucosidase acting optimally at pH 4.0 and 4.8; in Gaucher disease, only the former is deficient. Brief exposure of the leukocyte homogenate to pH 4.0 at room temperature results in irreversible inactivation of the pH 4.8 activity, while the activity at pH 4.0 remains unaffected. The more acidic isozyme is stimulated four- to fivefold by 0.2% sodium taurodeoxycholate (TDC) with a shift in the pH optimum to 5.0. The less… CONTINUE READING

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