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 | 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 | Comment | Organism | Structure |
---|---|---|---|
aminoethylbenzylsulfonyl fluoride | - |
Homo sapiens | |
additional information | resistant to inhibition by diisopropylfluorophosphate | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
lysosome | - |
Homo sapiens | 5764 | - |
Molecular Weight [Da] | Molecular Weight Maximum [Da] | Comment | Organism |
---|---|---|---|
48000 | - |
mature enzyme, SDS-PAGE | Homo sapiens |
68000 | - |
pro-enzyme, SDS-PAGE | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
glycoprotein | proteolytic modification | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Homo sapiens | - |
breast cancer cell | - |
Homo sapiens | - |
heart | - |
Homo sapiens | - |
lung fibroblast | - |
Homo sapiens | - |
placenta | - |
Homo sapiens | - |
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 | Comment | Organism |
---|---|---|
TPP I | - |
Homo sapiens |
pH Minimum | pH Maximum | Comment | Organism |
---|---|---|---|
2 | 5 | - |
Homo sapiens |
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 |