Glucosidase mutants

Cellobiase or / -glucosidase mutants can be detected by a number of plate assay techniques (1) esculin-ferric ammonium citrate, (2) cello-... 

Okuyama, H. Mori, K. Watanabe, A. and Kimura, S. Chiba. (2002). a-glucosidase mutant catalyzes a-glycosynthase -type reaction. Biosci Biotechnol Biochem, 66, 928-933. 

The observed change in the stereochemical behavior of the A. fecalis f3-glucosidase mutant acting on an aryl 0-D-glucoside11 does not arise solely from the elimination of assistance to a ft-direeled attack by water, but depends also on the creation of a new site of access located as to permit an on-directed attack by azide. One would wish to know the relative proportion... 

K., Kimura, A., and Chiba, S. (2002) Alpha-glucosidase mutant catalyzes "alpha-glycosynthase -type reaction. Biosci. Biotechnol. Biochem., 66, 928-933. 

Steet R, Chung S, Lee WS, Pine CW, Do H, Komfeld S. (2007) Selective action of the iminosugar isofagomine, a pharmacological chaperone for mutant forms of acid-beta-glucosidase. Biochem Pharmacol 73 1376-1383. 

Steel R, Chung S, Wustman B, Powe A, Do H, Komfeld SA. (2006) The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in gaucher fibroblasts by several mechanisms. Proc Natl Acad Sci USA 103 13813-13818. 

M. Czjzek, M. Cicek, V. Zamboni, D. R. Bevan, B. Henrissat, and A. Esim, The mechanism of substrate (aglycon) specificity in P-glucosidases is revealed by crystal structures of mutant maize p-glucosidase-DIMBOA, -DIMBOAGlc, and -dhurrin complexes, Proc. Natl. Acad. Sci. USA, 97 (2000)13555-13560. 

Gaucher disease is caused by a deficiency in lysosomal beta-glucosidase activity. Deoxynojirimycins are known inhibitors of several enzymes, which include beta-glucosidase. Several alkylated deoxynojirimycins were screened for correction of N370S Gaucher mutant lysosomal beta-glucosidase activity in an intact cell assay (10). A-(n-nonyl)deoxynojirimycin... 

Alpha-1-antitrypsin mutations are associated with early-onset emphysema and liver disease that results in early death (50). The Z-variant of the disease is the most common and is present in over 95% of cases. This variant has been shown to be rescued in vitro by treatment with proteasome inhibitors (50). Several chemical and pharmacological chaperones have also been shown to correct the secretion of the mutant protein, which include the glucosidase inhibitor castanospermine, as well as the mannosidase inhibitors kifunensine (Fig. 2b)... 

Another common mechanism of correction is ER glucosidase inhibition (Fig. 1, star 2), which prevents the recognition of misfolded proteins by calnexin and calreticulin and presumably allows them to escape the ERQC. Inhibition of glucosidase with castanospermine and miglustat were shown to correct the trafficking of deltaE508 CFTR and alpha-l-antitrypsin (51, 61). Whether this mechanism also works on other ER-retained mutants remains to be determined. 

Ida H, Eto Y, Ogawa S, Matsuzaki Y, Sawa M, Inoue T, Hi-gaki K, Nanba E, Ohno K, Suzuki Y. N-octyl-beta-valienamine up-regulates activity of F213I mutant beta-glucosidase in cultured... 

Marcus NY, Perknutter DH. Glucosidase and mannosidase inhibitors mediate increased secretion of mutant alphal antitrypsin Z. J. Biol. Chem. 2000 275 1987-1992. 

Sawker et al. reported that IV-nonyl-DNJ (97) is a potent inhibitor of lysosomal /3-glucosidase, with an IC50 value of 1 pM, and the addition of subinhibitory concentration (10 pM) of this compound to a fibroblast culture medium leads to a 2-fold increase in the mutant (N370S) enzyme activity [135]. Very recently, examination of a series of DNJ analogues on the resid-... 

MAL2 regulator of a-D-glucosidase synthesis some mutants are constitutive, others are non-utilizers 348-350... 

Maltose MALA MALS regulator of a-D-glucosidase synthesis constitutive for a-D-glucosidase synthesis and resistant to catabolite repression non-utilizing mutants have been found 348-351 349,352... 

MAL6 regulator of a-D-glucosidase and maltose carrier recessive mutants found (t) maltose non-utilizers, (ii) constitutive utilizers 349,353-355... 

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