By Reti L.
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Additional resources for Beta-Phenethylamines Manske and Holmes, The Alkaloids
Christomanou ( 2 9 ) claimed that the cofactor stimula ting β-glucosidase is also required for full activity of acid sphingomyelinase. The notion that one cofactor protein stimulates several enzymes is supported by a report of Konrad Sandhoff 22 Wenger et al. (30) who found that a non enzymic heat-stable protein from Gaucher spleen stimulated glucosylceramide-Bglucosidase, acid sphingomyelinase and galactosylceramideB-galactosidase. C. Activators for Ganglioside and G ^ degradation In 1973 Li and coworkers (31) found that a heat-stable, non dialyzable fraction obtained from human liver stimulated the degradation of ganglioside G ^ by crude hexosamini dase A preparation.
Recently a fourth variant of G gangliosidosis was un covered. The first case was originally described on clinical and biochemical grounds as AB variant (71), then as Pseudo AB variant (50). Li and coworkers (50) found that hexosaminidase A of that patient did not react with ganglioside G ^ i- the pre sence of G^""activator. Originally the authors suggested that an activator binding site of hexosaminidase A (hex A) is impaired as the underlying cause of the deficiency (39). Recent studies, however, demonstrate that hexosaminidase A ac 9 n 40 Konrad Sandhoff of that patient (40, 66) as well as that of a second patient (40) are completely inactive against the synthetic substrate p-nitrophenyl-N-acetyl-fi-D-glucosaminide-6-sulfate (PG6S) (Tab.
Controls were run with out activator protein and subtracted from the respective values. 2 w (g g) ganglioside G ^ ? (0 (• • ) ganglioside (Δ Δ ) ganglioside G ^ . D l a (A a s "A ) ganglioside G ^ ; O) glycolipid G A 2 ' 33 Activator Proteins for G l y c o l i p i d Degradation FIGURE 5. Model for the function of G 2 ~ i stimulatory factor for ganglioside G ^ degradation by h e x o s aminidase A, and as a glycosphingolipid transfer protein (48, 49) As a working hypothesis we speculate, furthermore, that hexosaminidase A has two different catalytic sites, one on the β-subunit and the other on the ot-subunit.
Beta-Phenethylamines Manske and Holmes, The Alkaloids by Reti L.