Studies on human N-acetyl-Beta-d-hexosaminidase C separated from neonatal brain

Human brain hexosaminidase C was separated from isoenzymes A and B by Sephadex G-200 gel filtration. Properties of the enzyme were studied, particularly its isoelectric-focusing profile, pI4.80. These findings indicate that hexosaminidase C is identical with the major residual component of Sandhoff fibroblasts with respect to substrate specificity, pI and activity pH optimum.