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Literature summary for 3.4.14.9 extracted from
- Tripeptidyl-peptidase I in health and disease (2006), Biol. Chem., 387, 1091-1099.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | mutations in tripeptidyl-peptidase I underlie the classic late-infantile form of neuronal ceroid lipofuscinoses (CLN2), the most common neurodegenerative disorders of childhood | Homo sapiens |
Protein Variants
| Protein Variants | Comment | Organism |
|---|---|---|
| C365R | decreased activity | Homo sapiens |
| E343L | decreased activity | Homo sapiens |
| G284V | decreased activity | Homo sapiens |
| G77R | decreased activity | Homo sapiens |
| I287N | decreased activity | Homo sapiens |
| N286S | decreased activity | Homo sapiens |
| P202L | decreased activity | Homo sapiens |
| P544S | decreased activity | Homo sapiens |
| P544S | demonstrates a normal polypeptide pattern on Western blots, enzyme activity, and lysosomal localization | Homo sapiens |
| Q422H | decreased activity | Homo sapiens |
| R127Q | decreased activity | Homo sapiens |
| R127Q | demonstrates a normal polypeptide pattern on Western blots, enzyme activity, and lysosomal localization | Homo sapiens |
| R206C | decreased activity | Homo sapiens |
| R447H | decreased activity | Homo sapiens |
| S475L | inactive | Homo sapiens |
| S475L | decreased activity | Homo sapiens |
| V277M | decreased activity | Homo sapiens |
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| aminoethylbenzylsulfonyl fluoride | - |
Homo sapiens |  |
| additional information | resistant to inhibition by diisopropylfluorophosphate | Homo sapiens |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| lysosome | - |
Homo sapiens | 5764 | - |
Molecular Weight [Da]
| Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
|---|---|---|---|
| 48000 | - |
mature enzyme, SDS-PAGE | Homo sapiens |
| 68000 | - |
pro-enzyme, SDS-PAGE | Homo sapiens |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Posttranslational Modification
| Posttranslational Modification | Comment | Organism |
|---|---|---|
| glycoprotein | proteolytic modification | Homo sapiens |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| brain | - |
Homo sapiens | - |
| breast cancer cell | - |
Homo sapiens | - |
| heart | - |
Homo sapiens | - |
| lung fibroblast | - |
Homo sapiens | - |
| placenta | - |
Homo sapiens | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| additional information | TPP I acts preferentially on small, unstructured oligopeptides of less than 5 kDa | Homo sapiens | ? | - |
? | |
| subunit C of mitochondrial ATP synthase + H2O | - |
Homo sapiens | ? | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| TPP I | - |
Homo sapiens |
pH Range
| pH Minimum | pH Maximum | Comment | Organism |
|---|---|---|---|
| 2 | 5 | - |
Homo sapiens |
pH Stability
| pH Stability | pH Stability Maximum | Comment | Organism |
|---|---|---|---|
| 2 | 5 | optimal activation occurs in the range pH 3.0-4.0, the proteolytic processing takes place in a wider pH range (2.0-5.0), at pH 3.0 and lower, TPP I is quickly inactivated, whereas the polypeptide generated at higher pH (4.5-5.0) possesses 6- and 13-aa N-terminal extensions and is inactive, the enzyme is unstable at alkaline and neutral pH | Homo sapiens |
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