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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
additional information
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-
ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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?
ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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?
ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
two-step reaction
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
GlyRS allelic variants are causally associated with the Charcot-Marie-Tooth disease, the most common genetic disorder of the peripheral nervous system
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
GlyRS mutations cause Charcot-Marie-Tooth peripheral neuropathies, the most common heritable disease of the peripheral nervous system, overview
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?
ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
mutations in the enzyme cause Charcot-Marie-Tooth disease type 2D, CMT2D, and distal spinal muscular atrophy type V, dSMA-V, axonal neuropathies characterized by a phenotype that is more severe in the upper extremities, in most cases, mutant GARS protein mislocalizes in neuronal cells
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
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the fusion protein catalyzes the aminoacylation of bovine tRNA
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
-
enzyme mutations with reduced enzyme activity cause distal spinal muscular atrophy type V or Charcot-Marie-Tooth type 2D, i.e. dSMAV/CMT2D
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?
ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
-
GlyRS mutations cause Charcot-Marie-Tooth peripheral neuropathies, at least 10 different mutant alleles, overview
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ATP + glycine + tRNAGly
AMP + diphosphate + glycyl-tRNAGly
-
tRNA binding site structure, and wild-type and S581L mutant active site structures, overview
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additional information
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structure-function analysis
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additional information
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structure-function analysis
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Brain Neoplasms
Intracellular glycine receptor function facilitates glioma formation in vivo.
Breast Neoplasms
A proteomic approach based on multiple parallel separation for the unambiguous identification of an antibody cognate antigen.
Carcinogenesis
Secreted human glycyl-tRNA synthetase implicated in defense against ERK-activated tumorigenesis.
Charcot-Marie-Tooth Disease
A novel adenoviral vector-mediated mouse model of Charcot-Marie-Tooth type 2D (CMT2D).
Charcot-Marie-Tooth Disease
A novel mutation in glycyl-tRNA synthetase caused Charcot-Marie-Tooth disease type 2D with facial and respiratory muscle involvement.
Charcot-Marie-Tooth Disease
A Novel Mutation of GARS in a Chinese Family With Distal Hereditary Motor Neuropathy Type V.
Charcot-Marie-Tooth Disease
A novel mutation of the glycyl-tRNA synthetase (GARS) gene associated with Charcot-Marie-Tooth type 2D in a Chinese family.
Charcot-Marie-Tooth Disease
Allele-specific RNA interference prevents neuropathy in Charcot-Marie-Tooth disease type 2D mouse models.
Charcot-Marie-Tooth Disease
Charcot-Marie-Tooth disease type 2D with a novel glycyl-tRNA synthetase gene (GARS) mutation.
Charcot-Marie-Tooth Disease
Charcot-Marie-Tooth-linked mutant GARS is toxic to peripheral neurons independent of wild-type GARS levels.
Charcot-Marie-Tooth Disease
Clinical and Genetic Features in a Series of Eight Unrelated Patients with Neuropathy Due to Glycyl-tRNA Synthetase (GARS) Variants.
Charcot-Marie-Tooth Disease
Cocrystal structures of glycyl-tRNA synthetase in complex with tRNA suggest multiple conformational states in glycylation.
Charcot-Marie-Tooth Disease
Further evidence for genetic heterogeneity of distal HMN type V, CMT2 with predominant hand involvement and Silver syndrome.
Charcot-Marie-Tooth Disease
GARS axonopathy: not every neuron's cup of tRNA.
Charcot-Marie-Tooth Disease
Glycyl tRNA synthetase mutations in Charcot-Marie-Tooth disease type 2D and distal spinal muscular atrophy type V.
Charcot-Marie-Tooth Disease
Neuromuscular junction maturation defects precede impaired lower motor neuron connectivity in Charcot-Marie-Tooth type 2D mice.
Charcot-Marie-Tooth Disease
Neuropilin 1 sequestration by neuropathogenic mutant glycyl-tRNA synthetase is permissive to vascular homeostasis.
Charcot-Marie-Tooth Disease
Novel GARS mutation presenting as autosomal dominant intermediate Charcot-Marie-Tooth disease.
Charcot-Marie-Tooth Disease
Two novel mutations of GARS in Korean families with distal hereditary motor neuropathy type V.
Dehydration
Hyperekplexia mutation of glycine receptors: decreased gating efficacy with altered binding thermodynamics.
Dermatomyositis
Autoantibodies to glycyl-transfer RNA synthetase in myositis. Association with dermatomyositis and immunologic heterogeneity.
Dermatomyositis
Primary structure and functional expression of human Glycyl-tRNA synthetase, an autoantigen in myositis.
Encephalomyelitis
Systemic delivery of human GlyR IgG antibody induces GlyR internalization into motor neurons of brainstem and spinal cord with motor dysfunction in mice.
Epilepsy
Allosteric Modulation of Glycine Receptors.
Epilepsy
Subunit-Specific Photocontrol of Glycine Receptors by Azobenzene-Nitrazepam Photoswitcher.
Epilepsy, Temporal Lobe
Allosteric Modulation of Glycine Receptors.
Hyperekplexia
A Missense Mutation A384P Associated with Human Hyperekplexia Reveals a Desensitization Site of Glycine Receptors.
Hyperekplexia
A Novel Glycine Receptor Variant with Startle Disease Affects Syndapin I and Glycinergic Inhibition.
Hyperekplexia
Anxiety and Startle Phenotypes in Glrb Spastic and Glra1 Spasmodic Mouse Mutants.
Hyperekplexia
Glycine receptor mutants of the mouse: what are possible routes of inhibitory compensation?
Hyperekplexia
Human Hyperekplexic Mutations in Glycine Receptors Disinhibit the Brainstem by Hijacking GABAA Receptors.
Hyperekplexia
Hyperekplexia mutation of glycine receptors: decreased gating efficacy with altered binding thermodynamics.
Hyperekplexia
Hyperekplexia mutation R271L of alpha1 glycine receptors potentiates allosteric interactions of nortropeines, propofol and glycine with [3H]strychnine binding.
Hyperekplexia
Hyperekplexia mutations of the glycine receptor unmask the inhibitory subsite for beta-amino-acids.
Hyperekplexia
Hyperekplexia phenotype of glycine receptor alpha1 subunit mutant mice identifies Zn(2+) as an essential endogenous modulator of glycinergic neurotransmission.
Hyperekplexia
Individual knock out of glycine receptor alpha subunits identifies a specific requirement of glra1 for motor function in zebrafish.
Hyperekplexia
Presynaptic glycine receptors as a potential therapeutic target for hyperekplexia disease.
Hyperekplexia
Subunit-Specific Photocontrol of Glycine Receptors by Azobenzene-Nitrazepam Photoswitcher.
Hyperekplexia
The role of charged residues in independent glycine receptor folding domains for intermolecular interactions and ion channel function.
Hyperekplexia
The role of tonic glycinergic conductance in cerebellar granule cell signalling and the effect of gain-of-function mutation.
Hyperglycinemia, Nonketotic
Glycine receptor mutants of the mouse: what are possible routes of inhibitory compensation?
Hyperglycinemia, Nonketotic
Inactivation of the glycine transporter 1 gene discloses vital role of glial glycine uptake in glycinergic inhibition.
Hypersensitivity
Modulation of glycine receptor single-channel conductance by intracellular phosphorylation.
Lipodystrophy
Familial asymmetric distal upper limb amyotrophy (Hirayama disease): report of a Greek family.
Lipodystrophy
Further evidence for genetic heterogeneity of distal HMN type V, CMT2 with predominant hand involvement and Silver syndrome.
Lipodystrophy
Relative contribution of mutations in genes for autosomal dominant distal hereditary motor neuropathies: a genotype-phenotype correlation study.
Lipodystrophy, Congenital Generalized
Familial asymmetric distal upper limb amyotrophy (Hirayama disease): report of a Greek family.
Lipodystrophy, Congenital Generalized
Further evidence for genetic heterogeneity of distal HMN type V, CMT2 with predominant hand involvement and Silver syndrome.
Lipodystrophy, Congenital Generalized
Relative contribution of mutations in genes for autosomal dominant distal hereditary motor neuropathies: a genotype-phenotype correlation study.
Lung Diseases, Interstitial
Antibodies to glycyl-transfer RNA synthetase in patients with myositis and interstitial lung disease.
Lung Diseases, Interstitial
Correlation of Antisynthetase Antibody Levels with Disease Course in a Patient with Interstitial Lung Disease and Elevated Muscle Enzymes.
Lupus Erythematosus, Systemic
Correlation of Antisynthetase Antibody Levels with Disease Course in a Patient with Interstitial Lung Disease and Elevated Muscle Enzymes.
Mitochondrial Diseases
Compound heterozygous mutations in glycyl-tRNA synthetase are a proposed cause of systemic mitochondrial disease.
Movement Disorders
Subunit-specific potentiation of recombinant glycine receptors by NV-31, a bilobalide-derived compound.
Muscle Spasticity
Altered potassium channel function in the superficial dorsal horn of the spastic mouse.
Muscle Spasticity
Anxiety and Startle Phenotypes in Glrb Spastic and Glra1 Spasmodic Mouse Mutants.
Muscle Spasticity
Despite GABAergic neurotransmission, GABAergic innervation does not compensate for the defect in glycine receptor postsynaptic aggregation in spastic mice.
Muscle Spasticity
Distinct physiological mechanisms underlie altered glycinergic synaptic transmission in the murine mutants spastic, spasmodic, and oscillator.
Muscular Atrophy
Effect of Electroacupuncture on the Expression of Glycyl-tRNA Synthetase and Ultrastructure Changes in Atrophied Rat Peroneus Longus Muscle Induced by Sciatic Nerve Injection Injury.
Muscular Atrophy, Spinal
An ENU-induced mutation in mouse glycyl-tRNA synthetase (GARS) causes peripheral sensory and motor phenotypes creating a model of Charcot-Marie-Tooth type 2D peripheral neuropathy.
Muscular Atrophy, Spinal
Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase, an enzyme underlying distal spinal muscular atrophy.
Muscular Atrophy, Spinal
Familial asymmetric distal upper limb amyotrophy (Hirayama disease): report of a Greek family.
Muscular Atrophy, Spinal
Glycyl tRNA synthetase mutations in Charcot-Marie-Tooth disease type 2D and distal spinal muscular atrophy type V.
Muscular Atrophy, Spinal
GRS defective axonal distribution as a potential contributor to distal spinal muscular atrophy type V pathogenesis in a new model of GRS-associated neuropathy.
Muscular Atrophy, Spinal
Mutant glycyl-tRNA synthetase (Gars) ameliorates SOD1(G93A) motor neuron degeneration phenotype but has little affect on Loa dynein heavy chain mutant mice.
Myoclonus
Systemic delivery of human GlyR IgG antibody induces GlyR internalization into motor neurons of brainstem and spinal cord with motor dysfunction in mice.
Myositis
Antibodies to glycyl-transfer RNA synthetase in patients with myositis and interstitial lung disease.
Myositis
Autoantibodies to glycyl-transfer RNA synthetase in myositis. Association with dermatomyositis and immunologic heterogeneity.
Myositis
Clinical Features and Cytokine Profile in Myositis Patients with Anti-EJ Autoantibodies Detected by a Novel Immunoprecipitation Assay.
Myositis
Primary structure and functional expression of human Glycyl-tRNA synthetase, an autoantigen in myositis.
Neoplasms
Extracellular vesicles derived from macrophages display glycyl-tRNA synthetase 1 and exhibit anti-cancer activity.
Neoplasms
Neddylation requires glycyl-tRNA synthetase to protect activated E2.
Neoplasms
Secreted human glycyl-tRNA synthetase implicated in defense against ERK-activated tumorigenesis.
Nervous System Diseases
Glycine Receptor Drug Discovery.
Nervous System Diseases
Hyperekplexia mutation R271L of alpha1 glycine receptors potentiates allosteric interactions of nortropeines, propofol and glycine with [3H]strychnine binding.
Neuralgia
Neuropathic pain model of peripheral neuropathies mediated by mutations of glycyl-tRNA synthetase.
Peripheral Nervous System Diseases
An Active Dominant Mutation of Glycyl-tRNA Synthetase Causes Neuropathy in a Charcot-Marie-Tooth 2D Mouse Model.
Peripheral Nervous System Diseases
An ENU-induced mutation in mouse glycyl-tRNA synthetase (GARS) causes peripheral sensory and motor phenotypes creating a model of Charcot-Marie-Tooth type 2D peripheral neuropathy.
Peripheral Nervous System Diseases
Charcot-Marie-Tooth-linked mutant GARS is toxic to peripheral neurons independent of wild-type GARS levels.
Peripheral Nervous System Diseases
Dispersed disease-causing neomorphic mutations on a single protein promote the same localized conformational opening.
Peripheral Nervous System Diseases
HDAC6 is a therapeutic target in mutant GARS-induced Charcot-Marie-Tooth disease.
Peripheral Nervous System Diseases
Neuropathic pain model of peripheral neuropathies mediated by mutations of glycyl-tRNA synthetase.
Seizures
Zinc enhances the inhibitory effects of strychnine-sensitive glycine receptors in mouse hippocampal neurons.
Spinal Muscular Atrophies of Childhood
Mutational analysis of glycyl-tRNA synthetase (GARS) gene in Hirayama disease.
Stiff-Person Syndrome
The GLRA1 missense mutation W170S associates lack of Zn2+ potentiation with human hyperekplexia.
Tinnitus
Activation of 5-HT2A/C receptor reduces glycine receptor-mediated currents in cultured auditory cortical neurons.
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C157R
the activity of the mutant enzyme is 62% of wild type
E71G/C157R
the activity of the mutant enzyme is 20% of wild type
G240R
the GARS mutation does not impair transcription or translation, modeled in yeast the mutation causes growth defects and impaired viability
H418R
modeled in yeast the mutation causes growth defects and impaired viability
L129P
modeled in yeast the mutation causes growth defects and impaired viability
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
R283A
the mutants retain 2% glycylation activity compared to the wild type enzyme
R283K
the mutants retain 20% glycylation activity compared to the wild type enzyme
R548A
the mutant shows strongly reduced activity compared to the wild type enzyme
R596Q
the mutation causes systemic mitochondrial disease
R67A
the mutant shows reduced activity compared to the wild type enzyme
S281A
the mutant shows reduced activity compared to the wild type enzyme
S635L
the mutation causes systemic mitochondrial disease
S91A
the mutant shows reduced activity compared to the wild type enzyme
T617A
the mutation reduces the enzymatic activity by more than 10fold
T631A
the mutant shows reduced activity compared to the wild type enzyme
A57V
-
found in a screen of 33 patients
P234KY
-
naturally occuring mutation in the catalytic domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
P244L
-
disease phenotype CMT2D
E71G
modeled in yeast the mutation causes growth defects and impaired viability
E71G
the mutant shows increased activity compared to the wild type enzyme
E71G
the mutant shows slightly elevated aminoacylation activity over wild type
G526R
modeled in yeast the mutation causes growth defects and impaired viability
G526R
naturally occuring mutation at the site for synthesis of glycyl-adenylate, G526 is a strictly conserved residue in the middle of motif 3, the mutant is inactive and shows tighter dimer interaction compared to the wild-type enzyme, long-range structural effects of the Charcot-Marie-Tooth disease-causing mutation in the human enzyme, overview
Y604F
the mutant shows strongly reduced activity compared to the wild type enzyme
Y604F
the mutation reduces the enzymatic activity by more than 10fold
D500N
-
naturally occuring mutation in the catalytic domain, the mutation lies in the disordered insertion III and causes Charcot-Marie-Tooth peripheral neuropathies
D500N
-
disease phenotype CMT2D/dSMA-V
E71G
-
naturally occuring mutation in the catalytic domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
E71G
-
disease phenotype CMT2D/dSMA-V
G240R
-
naturally occuring mutation in the catalytic domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
G240R
-
disease phenotype CMT2D
G526R
-
naturally occuring mutation in the catalytic domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
G526R
-
disease phenotype dSMA-V
G598A
-
naturally occuring mutation in the anticodon binding domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
H418R
-
naturally occuring mutation in the catalytic domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
H418R
-
disease phenotype dSMA-V
I280F
-
naturally occuring mutation in the catalytic domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
I280F
-
disease phenotype CMT2
L129P
-
naturally occuring mutation in the catalytic domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
L129P
-
disease phenotype dSMA-V
S581L
-
naturally occuring mutation in the anticodon binding domain, the mutation lies in a conserved region and causes Charcot-Marie-Tooth peripheral neuropathies
S581L
-
the naturally occuring dominant mutation to reduced aminoacylation activity and to motor nerve degeneration
S581L
-
disease phenotype lower limb predominant, CMT2
additional information
mutations in the enzyme cause Charcot-Marie-Tooth disease type 2D, CMT2D, and distal spinal muscular atrophy type V, dSMA-V, axonal neuropathies characterized by a phenotype that is more severe in the upper extremities, in most cases, mutant GARS protein mislocalizes in neuronal cells, and four of the five mutations show loss-of-function, GARS-associated granules occur in the neurite projections of cultured neurons and in the peripheral nerve axons of normal human tissue
additional information
-
mutations in the enzyme cause Charcot-Marie-Tooth disease type 2D, CMT2D, and distal spinal muscular atrophy type V, dSMA-V, axonal neuropathies characterized by a phenotype that is more severe in the upper extremities, in most cases, mutant GARS protein mislocalizes in neuronal cells, and four of the five mutations show loss-of-function, GARS-associated granules occur in the neurite projections of cultured neurons and in the peripheral nerve axons of normal human tissue
additional information
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-
additional information
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expression in Escherichia coli as a fusion protein with 13 kDa biotinylated tag with an apparent MW of 90 kDa
additional information
-
GlyRS mutations cause Charcot-Marie-Tooth peripheral neuropathies, at least 10 different mutant alleles, most catalytic-domain mutations are at the dimer interface, overview, mapping mutations onto human GlyRS crystal structure show them within a band encompassing both sides of the dimer interface, with two CMT-causing mutations being at sites that are complementary partners of a kissing contact across the dimer interface, the CMT phenotype does not correlate with aminoacylation activity, overview
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Freist, W.; Logan, D.T.; Gauss, D.H.
Glycyl-tRNA synthetase
Biol. Chem. Hoppe-Seyler
377
343-356
1996
Aliivibrio fischeri, Alcaligenes faecalis, Geobacillus stearothermophilus, Brevibacillus brevis, Bombyx mori, Bos taurus, Saccharomyces cerevisiae, Gallus gallus, Chlamydia trachomatis, Escherichia coli, eukaryota, Thermus thermophilus, Haemophilus influenzae, Homo sapiens, Staphylococcus aureus, Mus musculus, Mycoplasma genitalium, Rattus norvegicus, Salmonella enterica subsp. enterica serovar Typhimurium
brenda
Williams, J.; Osvath, S.; Khong, T.F.; Pearse.M.; Power, D.
Cloning, sequencing and bacterial expression of human glycine tRNA synthetase
Nucleic Acids Res.
23
1307-1310
1995
Homo sapiens
brenda
Ge, Q.; Trieu, E.P.; Targoff, I.N.
Primary structure and functional expression in human glycyl-tRNA synthetase, an autoantigen in myositis
J. Biol. Chem.
269
28790-28797
1994
Homo sapiens
brenda
Shiba, K.; Schimmel, P.; Motegi, H.; Noda, T.
Human glycyl-tRNA synthetase. Wide divergence of primary structure from bacterial counterpart and species-specific aminoacylation
J. Biol. Chem.
269
30049-30055
1994
Bombyx mori, Escherichia coli, Homo sapiens, Saccharomyces cerevisiae
brenda
Mudge, S.J.; Williams, J.H.; Eyre, H.J.; Sutherland, G.R.; Cowan, P.J.; Power, D.A.
Complex organisation of the 5'-end of the human glycine tRNA synthetase gene
Gene
209
45-50
1998
Homo sapiens (P41250), Homo sapiens
brenda
Xie, W.; Schimmel, P.; Yang, X.L.
Crystallization and preliminary X-ray analysis of a native human tRNA synthetase whose allelic variants are associated with Charcot-Marie-Tooth disease
Acta Crystallogr. Sect. F
62
1243-1246
2006
Homo sapiens (P41250), Homo sapiens
brenda
Cader, M.Z.; Ren, J.; James, P.A.; Bird, L.E.; Talbot, K.; Stammers, D.K.
Crystal structure of human wildtype and S581L-mutant glycyl-tRNA synthetase, an enzyme underlying distal spinal muscular atrophy
FEBS Lett.
581
2959-2964
2007
Homo sapiens
brenda
Antonellis, A.; Lee-Lin, S.Q.; Wasterlain, A.; Leo, P.; Quezado, M.; Goldfarb, L.G.; Myung, K.; Burgess, S.; Fischbeck, K.H.; Green, E.D.
Functional analyses of glycyl-tRNA synthetase mutations suggest a key role for tRNA-charging enzymes in peripheral axons
J. Neurosci.
26
10397-10406
2006
Homo sapiens (P41250), Homo sapiens
brenda
Nangle, L.A.; Zhang, W.; Xie, W.; Yang, X.L.; Schimmel, P.
Charcot-Marie-Tooth disease-associated mutant tRNA synthetases linked to altered dimer interface and neurite distribution defect
Proc. Natl. Acad. Sci. USA
104
11239-11244
2007
Homo sapiens
brenda
Xie, W.; Nangle, L.A.; Zhang, W.; Schimmel, P.; Yang, X.L.
Long-range structural effects of a Charcot-Marie-Tooth disease-causing mutation in human glycyl-tRNA synthetase
Proc. Natl. Acad. Sci. USA
104
9976-9981
2007
Homo sapiens (P41250), Homo sapiens
brenda
Dierick, I.; Baets, J.; Irobi, J.; Jacobs, A.; De Vriendt, E.; Deconinck, T.; Merlini, L.; Van den Bergh, P.; Rasic, V.M.; Robberecht, W.; Fischer, D.; Morales, R.J.; Mitrovic, Z.; Seeman, P.; Mazanec, R.; Kochanski, A.; Jordanova, A.; Auer-Grumbach, M.; Helderman-van den Enden, A.T.; Wokke, J.H.
Relative contribution of mutations in genes for autosomal dominant distal hereditary motor neuropathies: a genotype-phenotype correlation study
Brain
131
1217-1227
2008
Homo sapiens
brenda
Motley, W.W.; Talbot, K.; Fischbeck, K.H.
GARS axonopathy: not every neurons cup of tRNA
Trends Neurosci.
33
59-66
2010
Homo sapiens, Mus musculus
brenda
Blumen, S.; Drory, V.; Sadeh, M.; El-Ad, B.; Soimu, U.; Groozman, G.; Bouchard, J.; Goldfarb, L.
Mutational analysis of glycyl-tRNA synthetase (GARS) gene in Hirayama disease
Amyotroph. Lateral Scler.
11
237-239
2010
Homo sapiens
brenda
Andreev, D.E.; Hirnet, J.; Terenin, I.M.; Dmitriev, S.E.; Niepmann, M.; Shatsky, I.N.
Glycyl-tRNA synthetase specifically binds to the poliovirus IRES to activate translation initiation
Nucleic Acids Res.
40
5602-5614
2012
Homo sapiens
brenda
McMillan, H.J.; Schwartzentruber, J.; Smith, A.; Lee, S.; Chakraborty, P.; Bulman, D.E.; Beaulieu, C.L.; Majewski, J.; Boycott, K.M.; Geraghty, M.T.
Compound heterozygous mutations in glycyl-tRNA synthetase are a proposed cause of systemic mitochondrial disease
BMC Med. Genet.
15
36
2014
Homo sapiens (P41250)
brenda
Qin, X.; Hao, Z.; Tian, Q.; Zhang, Z.; Zhou, C.; Xie, W.
Cocrystal structures of glycyl-tRNA synthetase in complex with tRNA suggest multiple conformational states in glycylation
J. Biol. Chem.
289
20359-20369
2014
Homo sapiens (P41250), Homo sapiens
brenda
Deng, X.; Qin, X.; Chen, L.; Jia, Q.; Zhang, Y.; Zhang, Z.; Lei, D.; Ren, G.; Zhou, Z.; Wang, Z.; Li, Q.; Xie, W.
Large conformational changes of insertion 3 in human glycyl-tRNA synthetase (hGlyRS) during catalysis
J. Biol. Chem.
291
5740-5752
2016
Homo sapiens (P41250), Homo sapiens
brenda
Mo, Z.; Zhang, Q.; Liu, Z.; Lauer, J.; Shi, Y.; Sun, L.; Griffin, P.R.; Yang, X.L.
Neddylation requires glycyl-tRNA synthetase to protect activated E2
Nat. Struct. Mol. Biol.
23
730-737
2016
Homo sapiens (P41250), Homo sapiens
brenda
Chien, C.I.; Chen, Y.W.; Wu, Y.H.; Chang, C.Y.; Wang, T.L.; Wang, C.C.
Functional substitution of a eukaryotic glycyl-tRNA synthetase with an evolutionarily unrelated bacterial cognate enzyme
PLoS ONE
9
e94659
2014
Arabidopsis thaliana, Homo sapiens, Saccharomyces cerevisiae, Thermus thermophilus
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