Letter | Published:

Hexosaminidase-A and Hexosamini-dase-B: Studies in Tay-Sachs' and Sandhoff's Disease

Abstract

HUMAN tissues contain two isozymes of β-D-N-acetyl-hexos-aminidase1. Hexosaminidase-A (hex-A) is thermolabile and moves toward the anode on electrophoresis at pH 6.0, and hexosaminidase-B (hex-B) is relatively thermostable and moves toward the cathode. In Tay-Sachs' disease, which is found predominantly in Jewish populations, hex-A is absent but hex-B activity is increased2–5. In Sandhoff's disease, a variant of Tay-Sachs' disease, found mostly in non-Jewish populations, both enzyme activities are missing3,6. Thus, while it is apparent that there is a genetic relationship between hex-A and hex-B, the nature of this relationship has remained obscure. It has been suggested that hex-A may be derived from hex-B by the addition of neuraminic acid residues1,7.

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References

  1. 1

    Robinson, D., and Stirling, J. L., Biochem. J., 107, 321 (1968).

  2. 2

    Okada, S., and O'Brien, J. D., Science, 165, 698 (1969).

  3. 3

    Sandhoff, K., FEBS Lett., 4, 351 (1969).

  4. 4

    Hultberg, B., Lancet., ii, 1195 (1969).

  5. 5

    Kolodny, E. H., Brady, R. O., and Volk, B. W., Biochem. Biophys. Res. Comm., 37, 526 (1969).

  6. 6

    Sandhoff, K., Andrae, U., and Satzkewitz, H., Life Sci., 7, 283 (1968).

  7. 7

    Goldstone, A., Konecny, P., and Koenig, H., FEBS Lett., 13, 68 (1972).

  8. 8

    Carrol, D., and Robinson, D., Biochem. J., 126, 17p (1972).

  9. 9

    Srivastava, S. K., and Beutler, E., Biochem. Biophys. Res. Comm., 47, 753 (1972).

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