Beta-Phenethylamines Manske and Holmes, The Alkaloids - download pdf or read online

By Reti L.

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Additional resources for Beta-Phenethylamines Manske and Holmes, The Alkaloids

Sample text

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.

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Beta-Phenethylamines Manske and Holmes, The Alkaloids by Reti L.


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