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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) WB; IHC; ICC; IP.
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; IHC; ICC; IP.
MAD186Hu24 Monoclonal Antibody to Sucrase Isomaltase (SI) WB; IHC; ICC; IP.
MAD186Hu22 Monoclonal Antibody to Sucrase Isomaltase (SI) WB; IHC; ICC; IP.
MAD186Hu23 Monoclonal Antibody to Sucrase Isomaltase (SI) WB; IHC; ICC; IP.
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]