6.1.1.14: glycine-tRNA ligase

This is an abbreviated version!
For detailed information about glycine-tRNA ligase, go to the full flat file.

Word Map on EC 6.1.1.14

Reaction

ATP
+
glycine
+
tRNAGly
=
AMP
+
diphosphate
+
glycyl-tRNAGly

Synonyms

Glycyl-tRNA synthetase, Synthetase, glycyl-transfer ribonucleate, Glycyl-transfer ribonucleate synthetase, Glycyl-transfer RNA synthetase, Glycyl-transfer ribonucleic acid synthetase, Glycyl translase, GlyRS, More, Glycine--tRNA ligase, GARS, glycyl-tRNA synthetase 1, GRS1, GlyRS2, GlyRS1, glycyl tRNA synthetase

ECTree

     6 Ligases
         6.1 Forming carbon-oxygen bonds
             6.1.1 Ligases forming aminoacyl-tRNA and related compounds
                6.1.1.14 glycine-tRNA ligase

Engineering

Engineering on EC 6.1.1.14 - glycine-tRNA ligase

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PROTEIN VARIANTS
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
P61L
-
mutant enzymes with an altered amino acid binding site. Pro61Leu substitution in the alpha chain confers an elevation of the Km value for Gly, 25fold, and for ATP, 45fold, in the aminoacylation reaction, but only a minor pertubation of the Km for tRNA
R67A
the mutant shows reduced activity compared to the wild type enzyme
T617A
the mutation reduces the enzymatic activity by more than 10fold
R283A
the mutants retain 2% glycylation activity compared to the wild type enzyme
T631A
the mutant shows reduced activity compared to the wild type enzyme
P244L
-
disease phenotype CMT2D
S281A
the mutant shows reduced activity compared to the wild type enzyme
S91A
the mutant shows reduced activity compared to the wild type enzyme
D500N
R283K
the mutants retain 20% glycylation activity compared to the wild type enzyme
L129P
P234KY
-
naturally occuring mutation in the catalytic domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
G240R
I280F
H418R
G526R
S581L
G598A
R548A
the mutant shows strongly reduced activity compared to the wild type enzyme
A57V
-
found in a screen of 33 patients
S635L
the mutation causes systemic mitochondrial disease
R596Q
the mutation causes systemic mitochondrial disease
E71G/C157R
the activity of the mutant enzyme is 20% of wild type
C157R
the activity of the mutant enzyme is 62% of wild type
Y604F
Q640A
the mutation reduces the enzymatic activity by more than 10fold
Q675A
the mutation reduces the enzymatic activity by more than 10fold
Q675N
the mutation reduces the enzymatic activity by more than 10fold
Q82N
the mutant shows slightly increased activity compared to the wild type enzyme
GarsNmf249/+
-
mouse model, sensory and motor deficits, abnormal neuromuscular junction morphology, impaired nerve impule transmission, reduced nerve conduction velocities, loss of large diameter peripheral axons, life expectancy 6-8 weeks
GarsP278KY/+
-
mouse model, sensory and motor deficits, abnormal neuromuscular junction morphology, impaired nerve impule transmission, reduced nerve conduction velocities, loss of large diameter peripheral axons, life expectancy 6-8 weeks
GarsNmf249/Nmf249
-
mouse model, embryonic lethal
GarsXM256/+
-
mouse model, reduced GARS RNA levels, normal neuromuscular junction morphology, normal nerve conduction velocities, normal lifespan
GarsXM256/XM256
-
mouse model, embryonic lethal
GarsNmf249/XM256
-
mouse model, embryonic lethal
GarsC201R/+
-
mouse model, decreased grip strength, poor skilled motor function, increased total GARS protein at p15, reduction in large diameter axon in sciatic nerve, normal lifespan
GarsC201R/C201R
-
mouse model, reduced weight and viability, impaired limb movement, life expectancy 17 days
GarsC201R/XM256
-
mouse model, embryonic lethal
P552F
-
temperature-sensitive mutant grs1-1 shows altered substrate specificities, the mutant strain can be complemented by expression of the wild-type enzyme
additional information