sucrase isomaltase complex


Summary: An enzyme complex found in the brush border membranes of the small intestine. It is believed to be an enzyme complex with different catalytic sites. Its absence is manifested by an inherited disease called sucrase-isomaltase deficiency.

Top Publications

  1. Spodsberg N, Jacob R, Alfalah M, Zimmer K, Naim H. Molecular basis of aberrant apical protein transport in an intestinal enzyme disorder. J Biol Chem. 2001;276:23506-10 pubmed
    ..Obviously the O-glycans are not adequately recognized in the context of the mutant SI, most likely due to altered folding of the P-domain that ultimately affects the access of the O-glycans to a putative sorting element. ..
  2. Hunziker W, Spiess M, Semenza G, Lodish H. The sucrase-isomaltase complex: primary structure, membrane-orientation, and evolution of a stalked, intrinsic brush border protein. Cell. 1986;46:227-34 pubmed
    ..Following this is a high degree of homology between the isomaltase and sucrase portions (41% amino acid identity), indicating that pro-SI evolved by partial gene duplication. ..
  3. Jacob R, Zimmer K, Schmitz J, Naim H. Congenital sucrase-isomaltase deficiency arising from cleavage and secretion of a mutant form of the enzyme. J Clin Invest. 2000;106:281-7 pubmed
  4. Jacob R, Heine M, Alfalah M, Naim H. Distinct cytoskeletal tracks direct individual vesicle populations to the apical membrane of epithelial cells. Curr Biol. 2003;13:607-12 pubmed
    ..Our data further indicate that myosin 1A is the actin-associated motor protein that drives SAVs along actin filaments to the apical cell surface. ..
  5. Goda T. Regulation of the expression of carbohydrate digestion/absorption-related genes. Br J Nutr. 2000;84 Suppl 2:S245-8 pubmed
    ..These results suggest that the cis-elements of CE-LPH1 and SIF1 might be involved in the carbohydrate-induced increases of the transcription of LPH and SI, presumably through a change in the expression and/or binding activity of Cdx-2. ..
  6. Ouwendijk J, Moolenaar C, Peters W, Hollenberg C, Ginsel L, Fransen J, et al. Congenital sucrase-isomaltase deficiency. Identification of a glutamine to proline substitution that leads to a transport block of sucrase-isomaltase in a pre-Golgi compartment. J Clin Invest. 1996;97:633-41 pubmed
    ..This is the first report that characterizes a point mutation in the SI gene that is responsible for the transport incompetence of SI and for its retention between the ER and the Golgi. ..
  7. Wu G, Wang W, Traber P. Isolation and characterization of the human sucrase-isomaltase gene and demonstration of intestine-specific transcriptional elements. J Biol Chem. 1992;267:7863-70 pubmed
  8. Thompson Fawcett M, McC Mortensen N, Jewel D, Warren B. Use of a monoclonal antibody to sucrase-isomaltase for evaluation of the columnar cuff after stapled restorative proctocolectomy. Dis Colon Rectum. 2007;50:1428-35 pubmed
  9. Sander P, Alfalah M, Keiser M, Korponay Szabo I, Kovács J, Leeb T, et al. Novel mutations in the human sucrase-isomaltase gene (SI) that cause congenital carbohydrate malabsorption. Hum Mutat. 2006;27:119 pubmed
    ..All potential pathological alterations were present on one allele only. In six out of the 11 patients the phenotype of CSID could be explained by compound heterozygosity. ..

More Information


  1. Troelsen J, Olsen J, Møller J, Sjostrom H. An upstream polymorphism associated with lactase persistence has increased enhancer activity. Gastroenterology. 2003;125:1686-94 pubmed
    ..The discovery of a functional difference between the 2 alleles at position -13910 supports the notion that the molecular difference between lactase persistence and nonpersistence is caused by the mutation at position -13910. ..
  2. Bevilacqua M, Faniello M, Iovine B, Russo T, Cimino F, Costanzo F. Transcription factor NF-Y regulates differentiation of CaCo-2 cells. Arch Biochem Biophys. 2002;407:39-44 pubmed
  3. de Koning B, Lindenbergh Kortleve D, Pieters R, Rings E, Büller H, Renes I, et al. The effect of cytostatic drug treatment on intestine-specific transcription factors Cdx2, GATA-4 and HNF-1alpha in mice. Cancer Chemother Pharmacol. 2006;57:801-10 pubmed
  4. Uni Z. Research notes: Identification and isolation of chicken sucrase-isomaltase cDNA sequence. Poult Sci. 1998;77:140-4 pubmed
    ..Using our cDNA as a probe, Northern analysis revealed a transcript of approximately 6.0 kb in chicken jejunum and ileum tissues. ..
  5. Fitzgerald K, Bazar L, Avigan M. GATA-6 stimulates a cell line-specific activation element in the human lactase promoter. Am J Physiol. 1998;274:G314-24 pubmed
    ..These studies are consistent with an important role of an intestinal GATA binding protein in cell type-specific transactivation of the LPH promoter. ..
  6. Quaroni A, Tian J, Göke M, Podolsky D. Glucocorticoids have pleiotropic effects on small intestinal crypt cells. Am J Physiol. 1999;277:G1027-40 pubmed
    ..These effects are consistent with the activation or modulation of multiple genes important in the physiological function of absorptive villous cells but are probably not directly involved in the induction of cell differentiation. ..
  7. Takesue Y, Takesue S. Purification and characterization of alpha-glucosidase complex from the intestine of the frog, Rana japonica. Biochim Biophys Acta. 1996;1296:152-8 pubmed
    ..This implies that isomaltase is associated with maltase/glucoamylase to form alpha-glucosidase complex in the brush border membrane of the frog intestine. ..
  8. Tung J, Markowitz A, Silberg D, Traber P. Developmental expression of SI is regulated in transgenic mice by an evolutionarily conserved promoter. Am J Physiol. 1997;273:G83-92 pubmed
    ..These findings define a short SI gene promoter that contains cis-acting elements that are responsible for developmental and differentiation-dependent transcriptional regulation. ..
  9. Buts J, Duranton B, De Keyser N, Sokal E, Maernhout A, Raul F, et al. Premature stimulation of rat sucrase-isomaltase (SI) by exogenous insulin and the analog B-Asp10 is regulated by a receptor-mediated signal triggering SI gene transcription. Pediatr Res. 1998;43:585-91 pubmed
  10. Yamamoto H, Serizawa M, Thompson J, Sekiguchi J. Regulation of the glv operon in Bacillus subtilis: YfiA (GlvR) is a positive regulator of the operon that is repressed through CcpA and cre. J Bacteriol. 2001;183:5110-21 pubmed
    ..Collectively, our findings suggest that GlvR (the product of the glvR gene) is a positive regulator of the glv operon and that glucose exerts its effect via catabolite repression requiring both CcpA and cre. ..
  11. Reinke Y, Zimmer K, Naim H. Toxic peptides in Frazer's fraction interact with the actin cytoskeleton and affect the targeting and function of intestinal proteins. Exp Cell Res. 2009;315:3442-52 pubmed publisher
    ..The reduced levels of the disaccharidase SI in the BBM suggest a potential pathomechanism of carbohydrate malabsorption in CD. ..
  12. Traka M, Chambers K, Lund E, Goodlad R, Johnson I, Mithen R. Involvement of KLF4 in sulforaphane- and iberin-mediated induction of p21(waf1/cip1). Nutr Cancer. 2009;61:137-45 pubmed publisher
    ..These results suggest that induction of p21(waf1/cip1) by SF or IB may be partly mediated by KLF4 in some colon cancer cells and tissues. ..
  13. Ogura Y, Mochizuki K, Goda T. Induction of histone acetylation on the CRBPII gene in perinatal rat small intestine. Biochim Biophys Acta. 2007;1770:1289-96 pubmed
    ..These results suggest that CRBPII gene expression during the perinatal period is associated with abrupt acetylation of histones H3 and H4 followed by the binding of CBP/p300 and RXRalpha. ..
  14. Llor X, Pons E, Roca A, Alvarez M, Mane J, Fernández Bañares F, et al. The effects of fish oil, olive oil, oleic acid and linoleic acid on colorectal neoplastic processes. Clin Nutr. 2003;22:71-9 pubmed
    ..Fish oil and olive oil are capable of influencing crucial processes responsible for colorectal cancer development. COX-2 and Bcl-2 may be important mediators of some of these effects. ..
  15. Helmrath M, Fong J, Dekaney C, Henning S. Rapid expansion of intestinal secretory lineages following a massive small bowel resection in mice. Am J Physiol Gastrointest Liver Physiol. 2007;292:G215-22 pubmed
  16. Naim H, Joberty G, Alfalah M, Jacob R. Temporal association of the N- and O-linked glycosylation events and their implication in the polarized sorting of intestinal brush border sucrase-isomaltase, aminopeptidase N, and dipeptidyl peptidase IV. J Biol Chem. 1999;274:17961-7 pubmed
    ..The sorting of ApN implicates neither O-linked nor N-linked glycans and is driven most likely by carbohydrate-independent mechanisms. ..
  17. Schönert S, Buder T, Dahl M. Properties of maltose-inducible alpha-glucosidase MalL (sucrase-isomaltase-maltase) in Bacillus subtilis: evidence for its contribution to maltodextrin utilization. Res Microbiol. 1999;150:167-77 pubmed
    ..Furthermore, MalL expression varies temporally, showing a second induction in the stationary growth phase. ..
  18. Darimont C, Gradoux N, Cumin F, Baum H, De Pover A. Differential regulation of intestinal and liver fatty acid-binding proteins in human intestinal cell line (Caco-2): role of collagen. Exp Cell Res. 1998;244:441-7 pubmed
    ..It also shows that I-FABP is specifically regulated by collagen and that, under conditions optimal for their expression, both isoforms are modulated by metabolic factors. ..
  19. Bosse T, Fialkovich J, Piaseckyj C, Beuling E, Broekman H, Grand R, et al. Gata4 and Hnf1alpha are partially required for the expression of specific intestinal genes during development. Am J Physiol Gastrointest Liver Physiol. 2007;292:G1302-14 pubmed
    ..Together, these data demonstrate that specific intestinal genes have differential requirements for Gata4 and Hnf1alpha that are dependent on the developmental time frame in which they are expressed. ..
  20. Keller P, Semenza G, Shaltiel S. Phosphorylation of the N-terminal intracellular tail of sucrase-isomaltase by cAMP-dependent protein kinase. Eur J Biochem. 1995;233:963-8 pubmed
  21. Boudreau F, Zhu Y, Traber P. Sucrase-isomaltase gene transcription requires the hepatocyte nuclear factor-1 (HNF-1) regulatory element and is regulated by the ratio of HNF-1 alpha to HNF-1 beta. J Biol Chem. 2001;276:32122-8 pubmed
  22. Traber P, Wu G, Wang W. Novel DNA-binding proteins regulate intestine-specific transcription of the sucrase-isomaltase gene. Mol Cell Biol. 1992;12:3614-27 pubmed
    ..Taken together, these data have allowed the identification of novel DNA-binding proteins that play an important role in regulating intestine-specific transcription of the SI gene. ..
  23. Gorvel J, Ferrero A, Chambraud L, Rigal A, Bonicel J, Maroux S. Expression of sucrase-isomaltase and dipeptidylpeptidase IV in human small intestine and colon. Gastroenterology. 1991;101:618-25 pubmed
    ..In secretor blood group A humans, both the sucrase-isomaltase and the dipeptidylpeptidase IV have type 3 blood group A determinants. ..
  24. Gu N, Adachi T, Matsunaga T, Tsujimoto G, Ishihara A, Yasuda K, et al. HNF-1alpha participates in glucose regulation of sucrase-isomaltase gene expression in epithelial intestinal cells. Biochem Biophys Res Commun. 2007;353:617-22 pubmed
    ..These results suggest that HNF-1alpha participates in glucose regulation of SI gene expression. ..
  25. Moolenaar C, Ouwendijk J, Wittpoth M, Wisselaar H, Hauri H, Ginsel L, et al. A mutation in a highly conserved region in brush-border sucrase-isomaltase and lysosomal alpha-glucosidase results in Golgi retention. J Cell Sci. 1997;110 ( Pt 5):557-67 pubmed
  26. Chandrasena G, Osterholm D, Sunitha I, Henning S. Cloning and sequencing of a full-length rat sucrase-isomaltase-encoding cDNA. Gene. 1994;150:355-60 pubmed
    ..Given that this cDNA encodes the entire coding region of SI, it should be useful in elucidating the regulatory mechanisms of SI biosynthesis, localization and targeting during rat intestinal development and differentiation. ..
  27. Sklan D, Geyra A, Tako E, Gal Gerber O, Uni Z. Ontogeny of brush border carbohydrate digestion and uptake in the chick. Br J Nutr. 2003;89:747-53 pubmed
    ..It appears that both SI and SGLT-1 mRNA are expressed before hatch in the chick, but the ontogeny of expression is controlled by different mechanisms. ..
  28. Amit Romach E, Reifen R, Uni Z. Mucosal function in rat jejunum and ileum is altered by induction of colitis. Int J Mol Med. 2006;18:721-7 pubmed
    ..Involvement of the ileum and jejunum in TNBS-induced colitis may be related to the systemic reaction of the immune system and mucosa to colitis. ..
  29. Michaylira C, Ramocki N, Simmons J, Tanner C, McNaughton K, Woosley J, et al. Haplotype insufficiency for suppressor of cytokine signaling-2 enhances intestinal growth and promotes polyp formation in growth hormone-transgenic mice. Endocrinology. 2006;147:1632-41 pubmed
    ..Small variations in SOCS2 expression levels may significantly influence the outcome of therapeutic GH or acromegaly in intestine. ..
  30. Benoit Y, Paré F, Francoeur C, Jean D, Tremblay E, Boudreau F, et al. Cooperation between HNF-1alpha, Cdx2, and GATA-4 in initiating an enterocytic differentiation program in a normal human intestinal epithelial progenitor cell line. Am J Physiol Gastrointest Liver Physiol. 2010;298:G504-17 pubmed publisher
    ..These observations show that, in cooperation with Cdx2, HNF-1alpha acts as a key factor on human intestinal cells to trigger the onset of their functional differentiation program whereas GATA-4 appears to promote morphological changes. ..
  31. Ihara T, Tsujikawa T, Fujiyama Y, Ueyama H, Ohkubo I, Bamba T. Enhancement of brush border membrane peptidase activity in rat jejunum induced by starvation. Pflugers Arch. 2000;440:75-83 pubmed
  32. Lee S, Yu S, Nakayama J, Khoo K, Stone E, Fukuda M, et al. Core2 O-glycan structure is essential for the cell surface expression of sucrase isomaltase and dipeptidyl peptidase-IV during intestinal cell differentiation. J Biol Chem. 2010;285:37683-92 pubmed publisher
    ..These studies are the first to clearly identify functional mucin-type O-glycan structures modulating cell surface expression of SI and DPP-IV during the intestinal cell differentiation. ..
  33. Keiser M, Alfalah M, Pröpsting M, Castelletti D, Naim H. Altered folding, turnover, and polarized sorting act in concert to define a novel pathomechanism of congenital sucrase-isomaltase deficiency. J Biol Chem. 2006;281:14393-9 pubmed
    ..Altogether, the combined effects of the C635R mutation on the turnover rate, function, polarized sorting, and detergent solubility of SI constitute a unique and novel pathomechanism of CSID. ..
  34. Krasinski S, Van Wering H, Tannemaat M, Grand R. Differential activation of intestinal gene promoters: functional interactions between GATA-5 and HNF-1 alpha. Am J Physiol Gastrointest Liver Physiol. 2001;281:G69-84 pubmed
    ..Synergistic activation is a mechanism by which higher levels of tissue-specific expression might be attained by overlapping expression of specific transcription factors...
  35. Osborn M, Mazzoleni G, Santini D, Marrano D, Martinelli G, Weber K. Villin, intestinal brush border hydrolases and keratin polypeptides in intestinal metaplasia and gastric cancer; an immunohistologic study emphasizing the different degrees of intestinal and gastric differentiation in signet ring cell carcinomas. Virchows Arch A Pathol Anat Histopathol. 1988;413:303-12 pubmed
    ..In the keratin 7 positive tumours all tumour cells were keratin 7 positive. The keratin 8 antibody also reacted on routinely fixed specimens. Thus gastric carcinomas reveal different degrees of gastric and intestinal differentiation. ..
  36. Jacob R, Naim H. Apical membrane proteins are transported in distinct vesicular carriers. Curr Biol. 2001;11:1444-50 pubmed
  37. Takahashi J, Ogihara K, Naya Y, Kimura F, Itoh M, Iwama Y, et al. An in vitro assay system for antihyperlipidemic agents by evaluating lipoprotein profiles from human intestinal epithelium-like cells. 3 Biotech. 2013;3:213-218 pubmed publisher
    ..Pluronic L-81 at 1.0 ?g/ml inhibited TG contents in lipoprotein fractions from cells by 25.6 % and secretion was completely suppressed by the agent at 10 ?g/ml. ..
  38. Kitchen P, Fitzgerald A, Goodlad R, Barley N, Ghatei M, Legon S, et al. Glucagon-like peptide-2 increases sucrase-isomaltase but not caudal-related homeobox protein-2 gene expression. Am J Physiol Gastrointest Liver Physiol. 2000;278:G425-8 pubmed
    ..08). There was no change in Cdx-2 gene expression. Thus GLP-2 can maintain small intestinal morphology and function, but effects on gene expression are not mediated by gross changes in the level of the mRNA for the homeobox protein Cdx-2...
  39. Leeper L, McDonald M, Heath J, Henning S. Sucrase-isomaltase ontogeny: synergism between glucocorticoids and thyroxine reflects increased mRNA and no change in cell migration. Biochem Biophys Res Commun. 1998;246:765-70 pubmed
    ..Instead, we conclude that the synergism between the two hormones is due to greater accumulation of sucrase-isomaltase per epithelial cell. ..
  40. Hauri H, Roth J, Sterchi E, Lentze M. Transport to cell surface of intestinal sucrase-isomaltase is blocked in the Golgi apparatus in a patient with congenital sucrase-isomaltase deficiency. Proc Natl Acad Sci U S A. 1985;82:4423-7 pubmed
    ..The data imply that signals in sucrase-isomaltase that mediate its transfer from the endoplasmic reticulum to the Golgi apparatus differ from those mediating its transport from the Golgi apparatus to the cell surface. ..
  41. Mendes de Almeida J, Chaves P, Pereira A, Altorki N. Is Barrett's esophagus the precursor of most adenocarcinomas of the esophagus and cardia? A biochemical study. Ann Surg. 1997;226:725-33; discussion 733-5 pubmed
    ..These data show that BE and Adc of the esophagus and cardia have a similar phenotype and support the hypothesis that most of these tumors probably originate from preexisting BE. ..
  42. Fransen J, Hauri H, Ginsel L, Naim H. Naturally occurring mutations in intestinal sucrase-isomaltase provide evidence for the existence of an intracellular sorting signal in the isomaltase subunit. J Cell Biol. 1991;115:45-57 pubmed
    ..We conclude that structural features in the isomaltase region of pro-SI are required for transport and sorting of the sucrase-isomaltase complex. ..
  43. Goda T, Raul F, Gosse F, Koldovsky O. Effects of a high-protein, low-carbohydrate diet on degradation of sucrase-isomaltase in rat jejunoileum. Am J Physiol. 1988;254:G907-12 pubmed
    ..abstract truncated at 250 words) ..
  44. Suzuki T, Mochizuki K, Goda T. Histone H3 modifications and Cdx-2 binding to the sucrase-isomaltase (SI) gene is involved in induction of the gene in the transition from the crypt to villus in the small intestine of rats. Biochem Biophys Res Commun. 2008;369:788-93 pubmed publisher
  45. Honma K, Mochizuki K, Goda T. Carbohydrate/fat ratio in the diet alters histone acetylation on the sucrase-isomaltase gene and its expression in mouse small intestine. Biochem Biophys Res Commun. 2007;357:1124-9 pubmed
    ..These suggest that induction of SI gene by the diet rich in carbohydrate is associated with acetylation of histone H3 and H4 as well as binding of HNF-1 and Cdx-2 on SI gene. ..
  46. Picihard V, Berthois Y, Roccabianca M, Prevot C, Sarrazin M, Portugal H, et al. Concomitant cell growth and differentiation are dependent on erbB1 and integrin activation in an autonomously surviving colon adenocarcinoma: involvement of autocrine amphiregulin secretion. Anticancer Res. 2006;26:2769-83 pubmed
    ..It was concluded that in a colon adenocarcinoma, cell proliferation and differentiation can occur concomitantly and that these deregulated processes are controlled by autocrine secretion through the ErbB1/ERK1,2 and FAK pathways. ..
  47. Wild G, Searles L, Koski K, Drozdowski L, Begum Hasan J, Thomson A. Oral polyamine administration modifies the ontogeny of hexose transporter gene expression in the postnatal rat intestine. Am J Physiol Gastrointest Liver Physiol. 2007;293:G453-60 pubmed
    ..Thus, 3 days of oral polyamine administration induces the precocious maturation of glucose transporters in the postnatal rat small intestine, which may be mediated by alterations in ODC expression. ..
  48. Hauri H, Wacker H, Rickli E, Bigler Meier B, Quaroni A, Semenza G. Biosynthesis of sucrase-isomaltase. Purification and NH2-terminal amino acid sequence of the rat sucrase-isomaltase precursor (pro-sucrase-isomaltase) from fetal intestinal transplants. J Biol Chem. 1982;257:4522-8 pubmed
    ..A model is proposed for the mode of membrane assembly and the subsequent cleavage of pro-sucrase-isomaltase into its mature subunits. ..
  49. Schönert S, Buder T, Dahl M. Identification and enzymatic characterization of the maltose-inducible alpha-glucosidase MalL (sucrase-isomaltase-maltase) of Bacillus subtilis. J Bacteriol. 1998;180:2574-8 pubmed
    ..Insertional mutagenesis of malL resulted in a complete inactivation of the maltose-inducible alpha-glucosidase activity in crude protein extracts and a Mal- phenotype. ..
  50. Singh V, Pacheco I, Uversky V, Smith S, Macleod R, Jia Z. Intrinsically disordered human C/EBP homologous protein regulates biological activity of colon cancer cells during calcium stress. J Mol Biol. 2008;380:313-26 pubmed publisher
    ..These findings are discussed in the context of oligomerization of intrinsically disordered proteins as one of the mechanisms through which they exert their biological function. ..
  51. Bosse T, van Wering H, Gielen M, Dowling L, Fialkovich J, Piaseckyj C, et al. Hepatocyte nuclear factor-1alpha is required for expression but dispensable for histone acetylation of the lactase-phlorizin hydrolase gene in vivo. Am J Physiol Gastrointest Liver Physiol. 2006;290:G1016-24 pubmed
    ..These data indicate that HNF-1alpha is an activator of a subset of intestinal genes and induces these genes through an alternative mechanism in which it is dispensable for chromatin remodeling. ..
  52. Fang R, Olds L, Sibley E. Spatio-temporal patterns of intestine-specific transcription factor expression during postnatal mouse gut development. Gene Expr Patterns. 2006;6:426-32 pubmed
    ..Defining the spatio-temporal expression patterns for intestine-specific transcription factor genes contributes to investigation of the roles that factor gradients play in mediating gut development and differentiation. ..
  53. Houle V, Li W, Montgomery R, Grand R. mRNA localization in polarized intestinal epithelial cells. Am J Physiol Gastrointest Liver Physiol. 2003;284:G722-7 pubmed
    ..This study establishes a valuable model for the investigation of mRNA localization in intestinal epithelial cells. Mechanisms controlling asymmetrical distribution of intestinal mRNAs can be now be elucidated. ..