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Literature summary for 3.2.1.10 extracted from
- A glutamine to proline exchange at amino acid residue 1098 in sucrase causes a temperature-sensitive arrest of sucrase-isomaltase in the endoplasmic reticulum and cis-Golgi (2003), J. Biol. Chem., 278, 16310-16314.
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| transient expression of wild-type and mutant enzyme Q1098P in COS-1 cells | Homo sapiens |
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| additional information | congenital enzyme deficient mutant SI shows phenotype II featuring retention of the brush border enzyme in the cis-Golgi, which is blocked by additional mutantion Q1098P, expression of the resulting mutant SIQ/P in the enzyme deficient mutant cell line can partially restore correct folding, competent intracellular transport, and full enzyme activity at 20°C instead of 37°C due to evolved temperature-sensitivity of the mutant | Homo sapiens |
| Q1098P | congenital enzyme deficiency causes phenotype II featuring retention of the brush border enzyme in the cis-Golgi, which is blocked by additional mutantion Q1098P, expression of the resulting mutant SIQ/P in the enzyme deficient mutant cell line can partially restore correct folding, competent intracellular transport, and full enzyme activity at 20°C instead of 37°C due to evolved temperature-sensitivity of the mutant | Homo sapiens |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| endoplasmic reticulum | - |
Homo sapiens | 5783 | - |
| Golgi apparatus | - |
Homo sapiens | 5794 | - |
| membrane | - |
Homo sapiens | 16020 | - |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | Homo sapiens | phenotype II of congenital enzyme deficiency features the retention of the brush border protein in the cis-Golgi, this transport is blocked by substitution of Gln1098 by proline | ? | - |
? |  |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Posttranslational Modification
| Posttranslational Modification | Comment | Organism |
|---|---|---|
| glycoprotein | - |
Homo sapiens |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| intestine | - |
Homo sapiens | - |
Specific Activity [micromol/min/mg]
| Specific Activity Minimum [µmol/min/mg] | Specific Activity Maximum [µmol/min/mg] | Comment | Organism |
|---|---|---|---|
| 0.3 | - |
mutant SIQ/P, 37°C | Homo sapiens |
| 2.6 | - |
mutant SIQ/P, 20°C | Homo sapiens |
| 2.8 | - |
wild-type enzyme, 20°C | Homo sapiens |
| 3.1 | - |
wild-type enzyme, 37°C | Homo sapiens |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | phenotype II of congenital enzyme deficiency features the retention of the brush border protein in the cis-Golgi, this transport is blocked by substitution of Gln1098 by proline | Homo sapiens | ? | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| SI | - |
Homo sapiens |
| sucrase | - |
Homo sapiens |
| sucrase-isomaltase | - |
Homo sapiens |
Temperature Optimum [°C]
| Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|
| additional information | - |
mutant SIQ/P is temperature-sensitive with a permissive temperature for catalysis of 20°C, at 37°C intracellular degradation of the mutant protein occurs | Homo sapiens |
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