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GLYCOPROTEINS IN BRAIN TISSUE OF THE O‐VARIANT OF G M2 GANGLIOSIDOSIS
Author(s) -
Brunngraber E. G.,
Brown Barbara D.,
Aro Aurelia
Publication year - 1974
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/j.1471-4159.1974.tb12188.x
Subject(s) - gangliosidosis , mannose , hexosaminidase , glycoprotein , glycosaminoglycan , biochemistry , chemistry , enzyme , brain chemistry , microbiology and biotechnology , biology , endocrinology
— The dialysableglycopeptide preparation recovered from the glycoproteins in cerebral gray matter of a case of the O‐variant form of G M2 gangliosidosis contained four fold more N ‐acetylglucosamine and mannose than a similar preparation from normal gray matter. In the O‐variant form of G M2 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 G M2 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 G M2 gangliosidosis.