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Literature summary for 4.3.2.2 extracted from
- Is ZMP the toxic metabolite in Lesch-Nyhan disease? (2008), Med. Hypotheses, 71, 657-663.
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| 5-aminoimidazole-4-carboxamide riboside 5'-monophosphate | i.e. ZMP or AICAR, product inhibition, accumulation in the brain of Lesch-Nyhan disease patients induces inhibition of mitochondrial oxidative phosphorylation and adenylosuccinate lyase activity, followed by dowregulation of ATP levels and multifocal cell death in the cerebellum | Homo sapiens |  |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide | Homo sapiens | - |
5-aminoimidazole-4-carboxamide ribonucleotide + fumarate | ZMP accumulation in the brain of Lesch-Nyhan disease patients induces inhibition of mitochondrial oxidative phosphorylation and adenylosuccinate lyase activity, followed by dowregulation of ATP levels and multifocal cell death in the cerebellum. ZMP is an inhibitor of the bifunctional enzyme adenylosuccinate lyase, a deficiency of this enzyme causes psychomotor and mental retardation in humans, overview | ? | |
| additional information | Homo sapiens | ZMP is an activator of the AMP-activated protein kinase, AMPK, and is toxic in human B lymphocytes and neuroblastoma cell lines, physiological functions of ZMP, overview | ? | - |
? | |
| succinyladenosine monophosphate | Homo sapiens | - |
AMP + fumarate | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| B-lymphocyte | - |
Homo sapiens | - |
| neuroblastoma cell line | - |
Homo sapiens | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide | - |
Homo sapiens | 5-aminoimidazole-4-carboxamide ribonucleotide + fumarate | ZMP accumulation in the brain of Lesch-Nyhan disease patients induces inhibition of mitochondrial oxidative phosphorylation and adenylosuccinate lyase activity, followed by dowregulation of ATP levels and multifocal cell death in the cerebellum. ZMP is an inhibitor of the bifunctional enzyme adenylosuccinate lyase, a deficiency of this enzyme causes psychomotor and mental retardation in humans, overview | ? | |
| 5-aminoimidazole-4-(N-succinylcarboxamide) ribonucleotide | - |
Homo sapiens | 5-aminoimidazole-4-carboxamide ribonucleotide + fumarate | i.e. AICAR or ZMP | ? | |
| additional information | ZMP is an activator of the AMP-activated protein kinase, AMPK, and is toxic in human B lymphocytes and neuroblastoma cell lines, physiological functions of ZMP, overview | Homo sapiens | ? | - |
? | |
| succinyladenosine monophosphate | - |
Homo sapiens | AMP + fumarate | - |
? | |
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