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Sucrase Isomaltase (SI)

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Oligosaccharide Alpha-1,6-Glucosidase

Sucrase Isomaltase (SI)
CCC D186
HGNC 10856
MGI 1917233
PubMed 6762409
RGD 3675
UniProt P14410
Wiki SI
Congenital sucrase-isomaltase deficiency is an example of a disease in which mutation results in transport-incompetent molecules. sucrase-isomaltase is not transported to the brush border membrane but accumulates as a mannose-rich precursor in the endoplasmic reticulum, ER-Golgi intermediate compartment, and the cis-Golgi, where it is finally degraded.
The enzyme consists of 2 subunits which are synthesized as a single chain mannose-rich precursor. From studies of the cDNA, the human protein has 83% identity with the rabbit enzyme. In addition to the previously reported homology with lysosomal alpha-glucosidase, the sucrase and isomaltase subunits appear to be homologous to a yeast glucoamylase.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPD186Hu01 Recombinant Sucrase Isomaltase (SI) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAD186Hu01 Polyclonal Antibody to Sucrase Isomaltase (SI) IHC
LAD186Hu71 Biotin-Linked Polyclonal Antibody to Sucrase Isomaltase (SI) WB; IHC; ICC.
LAD186Hu81 FITC-Linked Polyclonal Antibody to Sucrase Isomaltase (SI) WB; IHC; ICC; IF.
MAD186Hu21 Monoclonal Antibody to Sucrase Isomaltase (SI) WB
MAD186Hu24 Monoclonal Antibody to Sucrase Isomaltase (SI) WB
MAD186Hu22 Monoclonal Antibody to Sucrase Isomaltase (SI) WB
MAD186Hu23 Monoclonal Antibody to Sucrase Isomaltase (SI) WB
Assay Kits SED186Hu ELISA Kit for Sucrase Isomaltase (SI) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Sucrase Isomaltase (SI) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Sucrase Isomaltase (SI) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Sucrase Isomaltase (SI) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Sucrase Isomaltase (SI) CLIA Kit Customized Service Offer
n/a ELISA Kit for Sucrase Isomaltase (SI) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Sucrase Isomaltase (SI) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Sucrase Isomaltase (SI) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Sucrase Isomaltase (SI) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Sucrase Isomaltase (SI) CLIA Kit Customized Service Offer
n/a ELISA Kit for Sucrase Isomaltase (SI) ELISA Kit Customized Service Offer
  1. "Sequence of the complete cDNA and the 5' structure of the human sucrase-isomaltase gene. Possible homology with a yeast glucoamylase."Biochem. J. 285:915-923(1992) [PubMed] [Europe PMC] [Abstract]
  2. "The DNA sequence, annotation and analysis of human chromosome 3." Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  4. "Isolation of a cDNA probe for a human jejunal brush-border hydrolase, sucrase-isomaltase, and assignment of the gene locus to chromosome 3."Gene 57:101-110(1987) [PubMed] [Europe PMC] [Abstract]
  5. "Expression of sucrase-isomaltase and dipeptidylpeptidase IV in human small intestine and colon."Gastroenterology 101:618-625(1991) [PubMed] [Europe PMC] [Abstract]
  6. "Phosphorylation of the N-terminal intracellular tail of sucrase-isomaltase by cAMP-dependent protein kinase."Eur. J. Biochem. 233:963-968(1995) [PubMed] [Europe PMC] [Abstract]
  7. "Structural basis for substrate selectivity in human maltase-glucoamylase and sucrase-isomaltase N-terminal domains."J. Biol. Chem. 285:17763-17770(2010) [PubMed] [Europe PMC] [Abstract]
  8. "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. 97:633-641(1996) [PubMed] [Europe PMC] [Abstract]
  9. "Congenital sucrase-isomaltase deficiency arising from cleavage and secretion of a mutant form of the enzyme."J. Clin. Invest. 106:281-287(2000) [PubMed] [Europe PMC] [Abstract]
  10. "Molecular basis of aberrant apical protein transport in an intestinal enzyme disorder."J. Biol. Chem. 276:23506-23510(2001) [PubMed] [Europe PMC] [Abstract]
  11. "Congenital sucrase-isomaltase deficiency because of an accumulation of the mutant enzyme in the endoplasmic reticulum."Gastroenterology 125:1678-1685(2003) [PubMed] [Europe PMC] [Abstract]
  12. "Novel mutations in the human sucrase-isomaltase gene (SI) that cause congenital carbohydrate malabsorption."Hum. Mutat. 27:119-119(2006) [PubMed] [Europe PMC] [Abstract]
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