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Literature summary for 6.1.1.21 extracted from

  • Abbott, J.A.; Meyer-Schuman, R.; Lupo, V.; Feely, S.; Mademan, I.; Oprescu, S.N.; Griffin, L.B.; Alberti, M.A.; Casasnovas, C.; Aharoni, S.; Basel-Vanagaite, L.; Zuechner, S.; De Jonghe, P.; Baets, J.; Shy, M.E.; Espinos, C.; Demeler, B.; Antonellis, A.; Francklyn, C.
    Substrate interaction defects in histidyl-tRNA synthetase linked to dominant axonal peripheral neuropathy (2018), Hum. Mutat., 39, 415-432 .
    View publication on PubMedView publication on EuropePMC
Search for more literature for 6.1.1.21

Protein Variants

Protein Variants Comment Organism
D175E the mutant with reduced aminoacylation activity is associated with Charcot-Marie-Tooth disease type 2W Homo sapiens
D364Y the mutant with reduced aminoacylation activity is associated with Charcot-Marie-Tooth disease type 2W Homo sapiens
P134H the mutant with reduced aminoacylation activity is associated with Charcot-Marie-Tooth disease type 2W Homo sapiens
R137Q the mutant with reduced aminoacylation activity is associated with Charcot-Marie-Tooth disease type 2W Homo sapiens
S356N the mutant with reduced aminoacylation activity is associated with Charcot-Marie-Tooth disease type 2W Homo sapiens
T132I the mutant with reduced aminoacylation activity is associated with Charcot-Marie-Tooth disease type 2W Homo sapiens
V155G the mutant with reduced aminoacylation activity is associated with Charcot-Marie-Tooth disease type 2W Homo sapiens
Y330C the mutant with reduced aminoacylation activity is associated with Charcot-Marie-Tooth disease type 2W Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.000199
-
 tRNAHis mutant enzyme S365N, at pH 7.5 and 25°C Homo sapiens
0.00033
-
 tRNAHis mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens
0.000782
-
 tRNAHis wild type enzyme, at pH 7.5 and 25°C Homo sapiens
0.000979
-
 tRNAHis mutant enzyme V155G, at pH 7.5 and 25°C Homo sapiens
0.008
-
 L-histidine wild type enzyme, at pH 7.5 and 25°C Homo sapiens
0.0108
-
 L-histidine mutant enzyme S365N, at pH 7.5 and 25°C Homo sapiens
0.0442
-
 ATP wild type enzyme, at pH 7.5 and 25°C Homo sapiens
0.2029
-
 L-histidine mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens
0.6872
-
 L-histidine mutant enzyme V155G, at pH 7.5 and 25°C Homo sapiens
1.763
-
 ATP mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + L-histidine + tRNAHis Homo sapiens
-
 AMP + diphosphate + L-histidyl-tRNAHis
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + L-histidine + tRNAHis
-
 Homo sapiens AMP + diphosphate + L-histidyl-tRNAHis
-
 ?

Subunits

Subunits Comment Organism
homodimer 2 * 118500, sedimentation velocity analysis Homo sapiens

Synonyms

Synonyms Comment Organism
HARS
-
 Homo sapiens
Histidyl-tRNA synthetase
-
 Homo sapiens

Temperature Stability [°C]

Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
51.7
-
 melting temperature Homo sapiens

Turnover Number [1/s]

Turnover Number Minimum [1/s] Turnover Number Maximum [1/s] Substrate Comment Organism Structure
0.3
-
 tRNAHis mutant enzyme S365N, at pH 7.5 and 25°C Homo sapiens
0.39
-
 L-histidine mutant enzyme S365N, at pH 7.5 and 25°C Homo sapiens
0.48
-
 ATP mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens
0.56
-
 tRNAHis mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens
0.74
-
 L-histidine mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens
2.08
-
 L-histidine mutant enzyme V155G, at pH 7.5 and 25°C Homo sapiens
3.05
-
 tRNAHis mutant enzyme V155G, at pH 7.5 and 25°C Homo sapiens
4.1
-
 L-histidine wild type enzyme, at pH 7.5 and 25°C Homo sapiens
5.4
-
 tRNAHis wild type enzyme, at pH 7.5 and 25°C Homo sapiens
5.8
-
 ATP wild type enzyme, at pH 7.5 and 25°C Homo sapiens

Cofactor

Cofactor Comment Organism Structure
ATP
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction mutations in histidyl-tRNA synthetase cause the dominant axonal peripheral neuropathy Charcot-Marie-Tooth disease type 2W Homo sapiens

kcat/KM [mM/s]

kcat/KM Value [1/mMs-1] kcat/KM Value Maximum [1/mMs-1] Substrate Comment Organism Structure
0.15
-
 ATP mutant enzyme S365N, at pH 7.5 and 25°C Homo sapiens
0.272
-
 ATP mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens
0.38
-
 ATP mutant enzyme V155G, at pH 7.5 and 25°C Homo sapiens
3
-
 L-histidine mutant enzyme V155G, at pH 7.5 and 25°C Homo sapiens
4
-
 L-histidine mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens
40
-
 L-histidine mutant enzyme S365N, at pH 7.5 and 25°C Homo sapiens
130
-
 ATP wild type enzyme, at pH 7.5 and 25°C Homo sapiens
500
-
 L-histidine wild type enzyme, at pH 7.5 and 25°C Homo sapiens
1500
-
 tRNAHis mutant enzyme S365N, at pH 7.5 and 25°C Homo sapiens
1700
-
 tRNAHis mutant enzyme Y330C, at pH 7.5 and 25°C Homo sapiens
3100
-
 tRNAHis mutant enzyme V155G, at pH 7.5 and 25°C Homo sapiens
6900
-
 tRNAHis wild type enzyme, at pH 7.5 and 25°C Homo sapiens