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Literature summary for 6.1.1.20 extracted from

  • Kartvelishvili, E.; Tworowski, D.; Vernon, H.; Moor, N.; Wang, J.; Wong, L.J.; Chrzanowska-Lightowlers, Z.; Safro, M.
    Kinetic and structural changes in HsmtPheRS, induced by pathogenic mutations in human FARS2 (2017), Protein Sci., 26, 1505-1516 .
    View publication on PubMedView publication on EuropePMC
Search for more literature for 6.1.1.20

Protein Variants

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 [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

Localization Comment Organism GeneOntology No. Textmining
mitochondrion
-
 Homo sapiens 5739
-

Natural Substrates/ Products (Substrates)

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

Organism UniProt Comment Textmining
Homo sapiens O95363
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + L-phenylalanine + tRNAPhe
-
 Homo sapiens AMP + diphosphate + L-phenylalanyl-tRNAPhe
-
 ?

Synonyms

Synonyms Comment Organism
FARS2
-
 Homo sapiens
Phenylalanyl-tRNA synthetase
-
 Homo sapiens
PheRS
-
 Homo sapiens

Turnover Number [1/s]

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

Cofactor Comment Organism Structure
ATP
-
 Homo sapiens

General Information

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 [mM/s]

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