Miglustat-induced intestinal carbohydrate malabsorption is due to the inhibition of α-glucosidases, but not β-galactosidases

Journal of Inherited Metabolic Disease

Volumn 35, Issue 6, 2012, Pages 949-954

  Amiri, Mahdi ^a   Naim, Hassan Y ^a  

^a UNIVERSITY OF VETERINARY MEDICINE HANNOVER   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA GLUCOSIDASE; BETA GALACTOSIDASE; LACTASE; MIGLUSTAT; OLIGO 1,6 GLUCOSIDASE; SUCRASE;

ARTICLE; CONTROLLED STUDY; ENZYME ACTIVITY; ENZYME INHIBITION; HUMAN; HUMAN TISSUE; HYDROLYSIS; INTESTINE BRUSH BORDER MEMBRANE; INTESTINE EPITHELIUM; MALABSORPTION; PROTEIN DEGRADATION; PROTEIN FOLDING;

1-DEOXYNOJIRIMYCIN; ALPHA-GLUCOSIDASES; BETA-GALACTOSIDASE; DIETARY CARBOHYDRATES; ENZYME INHIBITORS; GAUCHER DISEASE; HUMANS; INTESTINAL MUCOSA; KINETICS; MALABSORPTION SYNDROMES; MICROVILLI; NIEMANN-PICK DISEASE, TYPE C;

EID: 84867871154     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-012-9523-9     Document Type: Article

References (16)

1. Alfalah M, Jacob R, Preuss U, Zimmer K-P, Naim H, Naim HY (1999) O-linked glycans mediate apical sorting of human intestinal sucrase-isomaltase through association with lipid rafts. Curr Biol 9:593-596
2. Andersson U, Butters TD, Dwek RA, Platt FM (2000) N- butyldeoxygalactonojirimycin: a more selective inhibitor of glycosphingolipid biosynthesis than N-butyldeoxynojirimycin, in vitro and in vivo. Biochem Pharmacol 59:821-829
3. Belmatoug N, Burlina A, Giraldo P, Hendriksz CJ, Kuter DJ, Mengel E, Pastores GM (2011) Gastrointestinal disturbances and their management in miglustat-treated patients. J Inherit Metab Dis 34:991-1001
4. Cox T, Lachmann R, Hollak C, Aerts J, van Weely S, Hrebicek M, Platt F, Butters T, Dwek R, Moyses C, Gow I, Elstein D, Zimran A (2000) Novel oral treatment of Gaucher's disease with N-butyldeoxynojirimycin (OGT 918) to decrease substrate biosynthesis. Lancet 355:1481-1485
5. Elstein D, Dweck A, Attias D, Hadas-Halpern I, Zevin S, Altarescu G, Aerts J, van Weely S, Zimran A (2007) Oral maintenance clinical trial with miglustat for type I Gaucher disease: switch from or combination with intravenous enzyme replacement. Blood 110:2296-2301
6. Hauri HP, Sterchi EE, Bienz D, Fransen JA, Marxer A (1985) Expression and intracellular transport of microvillus membrane hydrolases in human intestinal epithelial cells. J Cell Biol 101:838-851
7. Lindner R, Naim HY (2009) Domains in biological membranes. Exp Cell Res 315:2871-2878
8. Maiuri L, Raia V, Potter J, Swallow D, Ho MW, Fiocca R, Finzi G, Cornaggia M, Capella C, Quaroni A, Auricchio S (1991) Mosaic pattern of lactase expression by villous enterocytes in human adult-type hypolactasia. Gastroenterology 100:359-369
9. Naim HY, Sterchi EE, Lentze MJ (1987a) Biosynthesis and maturation of lactase phlorizin hydrolase in the human small intestinal epithelial-cells. Biochem J 241:427-434
10. Naim HY, Sterchi EE, Lentze MJ (1987b) Kinetics of the processing of newly synthesized sucrase-isomaltase (SI) in organ-cultures of human intestinal biopsies. Pediatr Res 22:111-111
11. Naim HY, Sterchi EE, Lentze MJ (1988) Structure, biosynthesis, and glycosylation of human small intestinal maltase-glucoamylase. J Biol Chem 263:19709-19717
12. Quezada-Calvillo R, Sim L, Ao Z, Hamaker BR, Quaroni A, Brayer GD, Sterchi EE, Robayo-Torres CC, Rose DR, Nichols BL (2008) Luminal starch substrate brake on maltase-glucoamylase activity is located within the glucoamylase subunit. J Nutr 138:685-692
13. Robayo-Torres CC, Quezada-Calvillo R, Nichols BL (2006) Disaccharide digestion: clinical and molecular aspects. Clin Gastroenterol Hepatol 4:276-287
14. Rosenbaum AI, Maxfield FR (2011) Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches. J Neurochem 116:789-795
15. Schmitz J, Preiser H, Maestracci D, Ghosh BK, Cerda JJ, Crane RK (1973) Purification of the human intestinal brush border membrane. Biochim Biophys Acta-Biomembr 323:98-112
16. Treiber A, Morand O, Clozel M (2007) The pharmacokinetics and tissue distribution of the glucosylceramide synthase inhibitor miglustat in the rat. Xenobiotica 37:298-314