Application | Comment | Organism |
---|---|---|
medicine | enzyme may be useful as an adjunct to central nervous system-directed therapies and may be used as a starting point for modifications designed to improve brain delivery | Homo sapiens |
Cloned (Comment) | Organism |
---|---|
fragment corresponding to the entire coding region of PPT1 amplified from pCMV5-hPPT1, and cloned into XhoI sites in the polylinker region of expression vector pMSXND1, overexpressed in CHO cells. PPT1 injected intravenously into PPT1-deficient mice | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Purification (Comment) | Organism |
---|---|
by centrifugation, dialysis and gel filtration | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
additional information | lymphoblasts from patients with infantile neuronal ceroid lipofuscinosis, deficient of PPT1 | Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-methylumbelliferyl 6-S-palmitoyl-6-thio-beta-D-glucoside + H2O | - |
Homo sapiens | 4-methylumbelliferyl 6-thio-beta-D-glucoside + palmitate | - |
? |
Synonyms | Comment | Organism |
---|---|---|
palmitoyl-protein thioesterase-1 | - |
Homo sapiens |
PPT1 | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
physiological function | when injected intravenously into PPT1-deficient mice, the clearance of recombinant PPT1 from plasma is rapid, with a half-life of 10 min. Most of the injected dose is distributed to the kidney and liver and potentially corrective levels are also observed in heart, lung and spleen. Brain uptake is minimal. The enzyme is largely mannose 6-phosphorylated and takes up rapidly by PPT1-deficient immortalized lymphoblasts derived from infantile neuronal ceroid lipofuscinosis patients | Homo sapiens |