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Literature summary for 3.7.1.2 extracted from
- Slow-onset inhibition of fumarylacetoacetate hydrolase by phosphinate mimics of the tetrahedral intermediate: kinetics, crystal structure and pharmacokinetics (2007), Biochem. J., 402, 251-260.
Application
| Application | Comment | Organism |
|---|---|---|
| medicine | hereditary tyrosinaemia type 1 results from deficiency of fumarylacetoacetase | Homo sapiens |
| medicine | hereditary tyrosinaemia type 1 results from deficiency of fumarylacetoacetase | Mus musculus |
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| for expression in Escherichia coli | Homo sapiens |
| for expression in Escherichia coli | Mus musculus |
Crystallization (Commentary)
| Crystallization (Comment) | Organism |
|---|---|
| a high-resolution crystal structure of the FAH-CEHPOBA complex is soved at 1.35 A | Mus musculus |
Inhibitors
| Inhibitors | Comment | Organism | Structure |
|---|---|---|---|
| 3-[(3-carboxy-2-oxopropyl)hydroxyphosphinyl]acrylate | COPHPAA | Homo sapiens |  |
| 3-[(3-carboxy-2-oxopropyl)hydroxyphosphinyl]acrylate | COPHPAA | Mus musculus | |
| 4-[(2-carboxyethyl)-hydroxyphosphinyl]-3-oxobutyrate | CEHPOBA | Homo sapiens | |
| 4-[(2-carboxyethyl)-hydroxyphosphinyl]-3-oxobutyrate | CEHPOBA | Mus musculus | |
Natural Substrates/ Products (Substrates)
| Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 4-fumarylacetoacetate + H2O | Homo sapiens | - |
acetoacetate + fumarate | - |
? | |
| 4-fumarylacetoacetate + H2O | Mus musculus | - |
acetoacetate + fumarate | - |
? | |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | - |
- |
- |
| Mus musculus | P35505 | - |
- |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
- |
Homo sapiens |
- |
Mus musculus |
Source Tissue
| Source Tissue | Comment | Organism | Textmining |
|---|---|---|---|
| liver | - |
Mus musculus | - |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| 4-fumarylacetoacetate + H2O | - |
Homo sapiens | acetoacetate + fumarate | - |
? | |
| 4-fumarylacetoacetate + H2O | - |
Mus musculus | acetoacetate + fumarate | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| FAA hydrolase | - |
Homo sapiens |
| FAA hydrolase | - |
Mus musculus |
| FAH | - |
Homo sapiens |
| FAH | - |
Mus musculus |
| fumarylacetoacetase | - |
Homo sapiens |
| fumarylacetoacetase | - |
Mus musculus |
| fumarylacetoacetate hydrolase | - |
Homo sapiens |
| fumarylacetoacetate hydrolase | - |
Mus musculus |
| mFAH | - |
Mus musculus |
pH Optimum
| pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|
| 7 | - |
activity assay | Homo sapiens |
| 7 | - |
activity assay | Mus musculus |
Ki Value [mM]
| Ki Value [mM] | Ki Value maximum [mM] | Inhibitor | Comment | Organism | Structure |
|---|---|---|---|---|---|
| 0.000012 | - |
3-[(3-carboxy-2-oxopropyl)hydroxyphosphinyl]acrylate | - |
Homo sapiens | |
| 0.000041 | - |
4-[(2-carboxyethyl)-hydroxyphosphinyl]-3-oxobutyrate | - |
Homo sapiens | |
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