Cloned (Comment) | Organism |
---|---|
PPT1-deficient fibroblasts stably transfected with cDNA encoding PPT1 | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P50897 | - |
- |
Mus musculus | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
fibroblast | normal fibroblasts and infantile neuronal ceroid lipofuscinosis fibroblasts, deficient of PPT1 | Homo sapiens | - |
additional information | Ppt1/Cln1-deficient cells | Mus musculus | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-methylumbelliferyl 6-S-palmitoyl-6-thio-beta-D-glucoside + H2O | - |
Mus musculus | 4-methylumbelliferyl 6-thio-beta-D-glucoside + palmitate | - |
? | |
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 | - |
Mus musculus |
palmitoyl protein thioesterase 1 | - |
Homo sapiens |
PPT1 | - |
Mus musculus |
PPT1 | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | PPT1 deficient cells are partially resistant to tumor necrosis factor-induced cell death (57-75% cell viability versus 15-30% for control fibroblasts), tumor necrosis factor-initiated proteolytic cleavage of caspase-8, Bid and caspase-3, as well as cytochrome c release is strongly attenuated. Activation of p42/p44 mitogen-activated protein kinase and of transcription factor NF-kappaB by tumor necrosis factor, and induction of cell death by staurosporine or chemotherapeutic drugs in infantile neuronal ceroid lipofuscinosis cells are unaffected by PPT1 deficiency | Homo sapiens |
malfunction | resistance to tumor necrosis factor-induced apoptosis in embryonic fibroblasts derived from Ppt1/Cln1-deficient mice but not from mice with a targeted deletion of Cln3 or Cln5 (murine models of ceroid lipofuscinosis) | Mus musculus |
physiological function | role of PPT1 and, likely, protein depalmitoylation in the regulation of tumor necrosis factor-induced apoptosis | Mus musculus |
physiological function | role of PPT1 and, likely, protein depalmitoylation in the regulation of tumor necrosis factor-induced apoptosis. PPT1 is involved in cell death triggered by death receptors but not receptor-independent cytotoxic agents. Expression of PPT1 cDNA in infantile neuronal ceroid lipofuscinosis cells restores the tumor necrosis factor sensitivity | Homo sapiens |