| Product Name | Human Sucrase-isomaltase, intestinal (SI) ELISA Kit |
|---|---|
| Description | 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. This assay has high sensitivity and excellent specificity for detection of Human SI. No significant cross-reactivity or interference between Human SI and analogues was observed. |
| Synonyms | MGC131621, MGC131622, Oligosaccharide alpha-1,6-glucosidase|sucrase-isomaltase |
| Method | Sandwich ELISA |
| Detection Range | 0.312-20 ng/mL |
| Sensitivity | 0.107 ng/mL |
| Reactivity | Human |
| Sample Types | Serum, Plasma, Other biological fluids. |
| Background |
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. |
| Supplier | Abebio |
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