Protein Variants | Comment | Organism |
---|---|---|
D289Y | the mutant shows 52% of wild type activity | Homo sapiens |
G273S | inactive | Homo sapiens |
H123P | inactive | Homo sapiens |
H99D | the mutant shows 2.7% of wild type activity | Homo sapiens |
P49A | the mutant shows 78% of wild type activity | Homo sapiens |
R117G | the mutant shows 2.1% of wild type activity | Homo sapiens |
R383C | the mutant shows 43% of wild type activity | Homo sapiens |
R387Q | the mutant shows 86% of wild type activity | Homo sapiens |
T210M | the mutant shows 140% of wild type activity | Homo sapiens |
KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.00036 | - |
tRNAPhe | mutant enzyme T210M, at pH 8.5 and 37°C | Homo sapiens | |
0.001 | - |
tRNAPhe | mutant enzyme D289Y, at pH 8.5 and 37°C | Homo sapiens | |
0.0011 | - |
tRNAPhe | mutant enzyme P49A, at pH 8.5 and 37°C | Homo sapiens | |
0.0012 | - |
tRNAPhe | wild type enzyme, at pH 8.5 and 37°C | Homo sapiens | |
0.0012 | - |
tRNAPhe | mutant enzyme R387Q, at pH 8.5 and 37°C | Homo sapiens | |
0.0021 | - |
tRNAPhe | mutant enzyme R383C, at pH 8.5 and 37°C | Homo sapiens | |
0.0024 | - |
L-phenylalanine | wild type enzyme, at pH 8.5 and 37°C | Homo sapiens | |
0.0056 | - |
L-phenylalanine | mutant enzyme H99D, at pH 8.5 and 37°C | Homo sapiens | |
0.012 | - |
L-phenylalanine | mutant enzyme R117G, at pH 8.5 and 37°C | Homo sapiens |
Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
mitochondrion | - |
Homo sapiens | 5739 | - |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + L-phenylalanine + tRNAPhe | Homo sapiens | - |
AMP + diphosphate + L-phenylalanyl-tRNAPhe | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | O95363 | - |
- |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + L-phenylalanine + tRNAPhe | - |
Homo sapiens | AMP + diphosphate + L-phenylalanyl-tRNAPhe | - |
? |
Synonyms | Comment | Organism |
---|---|---|
FARS2 | - |
Homo sapiens |
Phenylalanyl-tRNA synthetase | - |
Homo sapiens |
PheRS | - |
Homo sapiens |
Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
0.012 | - |
L-phenylalanine | mutant enzyme H99D, at pH 8.5 and 37°C | Homo sapiens | |
0.02 | - |
L-phenylalanine | mutant enzyme R117G, at pH 8.5 and 37°C | Homo sapiens | |
0.07 | - |
tRNAPhe | mutant enzyme R383C, at pH 8.5 and 37°C | Homo sapiens | |
0.08 | - |
tRNAPhe | mutant enzyme D289Y, at pH 8.5 and 37°C | Homo sapiens | |
0.14 | - |
tRNAPhe | mutant enzyme P49A, at pH 8.5 and 37°C | Homo sapiens | |
0.14 | - |
tRNAPhe | mutant enzyme R387Q, at pH 8.5 and 37°C | Homo sapiens | |
0.19 | - |
L-phenylalanine | wild type enzyme, at pH 8.5 and 37°C | Homo sapiens | |
0.19 | - |
tRNAPhe | wild type enzyme, at pH 8.5 and 37°C | Homo sapiens | |
0.22 | - |
tRNAPhe | mutant enzyme T210M, at pH 8.5 and 37°C | Homo sapiens |
Cofactor | Comment | Organism | Structure |
---|---|---|---|
ATP | - |
Homo sapiens |
General Information | Comment | Organism |
---|---|---|
malfunction | pathogenic variants of the FARS2 gene, encoding the human mitochondrial phenylalanine-tRNA synthetase lead to spastic paraplegia or fatal infantile Alpers encephalopathy | Homo sapiens |
kcat/KM Value [1/mMs-1] | kcat/KM Value Maximum [1/mMs-1] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|
1.7 | - |
L-phenylalanine | mutant enzyme R117G, at pH 8.5 and 37°C | Homo sapiens | |
2.2 | - |
L-phenylalanine | mutant enzyme H99D, at pH 8.5 and 37°C | Homo sapiens | |
7.2 | - |
tRNAPhe | mutant enzyme R383C, at pH 8.5 and 37°C | Homo sapiens | |
8.7 | - |
tRNAPhe | mutant enzyme D289Y, at pH 8.5 and 37°C | Homo sapiens | |
14.3 | - |
tRNAPhe | mutant enzyme R387Q, at pH 8.5 and 37°C | Homo sapiens | |
23.3 | - |
tRNAPhe | mutant enzyme T210M, at pH 8.5 and 37°C | Homo sapiens | |
80 | - |
L-phenylalanine | wild type enzyme, at pH 8.5 and 37°C | Homo sapiens | |
125 | - |
tRNAPhe | mutant enzyme P49A, at pH 8.5 and 37°C | Homo sapiens | |
160 | - |
tRNAPhe | wild type enzyme, at pH 8.5 and 37°C | Homo sapiens |