By John A. Barranger
Molecular foundation of Lysosomal garage Disorders
summary: Molecular foundation of Lysosomal garage problems
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Interaction was strongest with ganglioside G , indicating that the carbohydrate recognition site of the G 2 " " displays some specificity for the glycolipid substrate. The water-soluble complexes are obviously formed by extracting the glycolipid from membranes or from micellar structures. This concept is supported by the fact that the purified G 2 ~ / a ganglioside G ^ binding protein, can act as a glycolipid transfer protein in the absence of hexosaminidase A. Under suitable in vitro conditions, ganglioside G ^ extracted by the activator from G«-loaded liposomes and transferred to acceptor liposomes (Fig.
4 - 5 ) . The accumulation of ganglioside G and of the neutral glycolipid G in the nervous tissue of patients afflicted with variant Ο of G ^ gangliosidosis can be understood when assuming that the minor isoenzyme β-hexosaminidase S which is still present in these tissues (42) cannot interact with the activator-lipid complex. (This was indeed observed in some preliminary experiments /Conzelmann and Sandhoff, una c t M 9 v a t o r 38 Konrad Sandhoff published^)· Storage of the same glycolipids in variant Β of G gangliosidosis is explained by the isoenzyme specificity or the activator protein: The G^-activator promotes glyco lipid degradation by β-hexosaminidase A but does hardly inter act with the Β isoenzyme (48) which therefore cannot attack the glycolipid substrates.
Neurochem. 12:210 (1981). 31. , Wan, C . , J. Biol. Chem. 248:7512 (1973). 32. C. , J. Biol. Chem. 251:1159 (1976). 33. , Dissertation, Amsterdam University (1978). 47 Activator Proteins for Glycolipid Degradation 3 4 . , J. Biol. Chem. 2 5 4 : 1 0 5 9 2 (1979). 3 5 . , J. Biol. , ( 1 9 8 3 ) in press. 3 6 . , J. Biol. Chem. 256:6234 (1981). 3 7 . Conzelmann, E. , Proc. Natl. Acad. Sci. USA 7 5 : 3 9 7 9 (1978). 3 8 . , Pediatr. Res. 1 6 : 2 1 7 (1982). 3 9 . , Li, Υ. , J. 40: 168 (1983). 4 0 .