GLYCOPROTEINS IN BRAIN TISSUE OF THE O-VARIANT OF GM2 GANGLIOSIDOSIS |
| |
Authors: | E. G. Brunngraber Barbara D. Brown Aurelia Aro |
| |
Affiliation: | Research Department, Illinois State Psychiatric Institute, 1601 West Taylor Street, Chicago, IL 60612, U.S.A. |
| |
Abstract: | Abstract— The dialysableglycopeptide preparation recovered from the glycoproteins in cerebral gray matter of a case of the O-variant form of GM2 gangliosidosis contained four fold more N -acetylglucosamine and mannose than a similar preparation from normal gray matter. In the O-variant form of GM2 gangliosidosis, the enzymes β - N -acetylhexosaminidases A and B are missing. A three- and four-fold elevation, respectively, of N -acetylglucosamine and mannose in the dialysable glycopeptide preparation from a case of Tay-Sachs disease (B-variant form of GM2 gangliosidosis) was noted. The B-variant lacks hexosaminidase A but has ample supplies of hexosaminidase B. The brain level of glycosaminoglycans was not affected in the O- and B-variant forms of GM2 gangliosidosis. |
| |
Keywords: | |
|
|