6.1.1.9: valine-tRNA ligase

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For detailed information about valine-tRNA ligase, go to the full flat file.

Reaction

Synonyms

G7a, glp-4, mitochondrial valyl tRNA synthetase, More, MTTV, Os03g0694900, OsValRS2, Synthetase, valyl-transfer ribonucleate, Val-tRNA synthetase, Valine transfer ribonucleate ligase, Valine translase, Valine--tRNA ligase, ValRS, ValRS1, ValRS2, valS, valyl aminoacyl tRNA synthetase, Valyl transfer ribonucleic acid synthetase, Valyl-transfer ribonucleate synthetase, Valyl-transfer RNA synthetase, Valyl-tRNA ligase, Valyl-tRNA synthetase, valyl-tRNAsynthetase, VARS, VARS-2, Vas1, Vas2, white panicle1

ECTree

     6 Ligases

         6.1 Forming carbon-oxygen bonds

             6.1.1 Ligases forming aminoacyl-tRNA and related compounds

                6.1.1.9 valine-tRNA ligase

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Results	in table
4	Activating Compound
58066	AA Sequence
1	Application
1	CAS Registry Number
15	Cloned(Commentary)
16	Cofactor
5	Crystallization (Commentary)
38	Disease/ Diagnostics
1	Expression
15	General Information
3	General Stability
16	Inhibitors
4	kcat/KM [mM/s]
5	Ki Value [mM]
72	KM Value [mM]
28	Localization
22	Metals/Ions
25	Molecular Weight [Da]
21	Natural Substrates/ Products (Substrates)
202	Organism
4	Pathway
9	PDB ID
13	pH Optimum
2	pH Range
29	Protein Variants
18	Purification (Commentary)
6	Reaction
18	Reaction Type
63	Reference
11	Source Tissue
12	Specific Activity [micromol/min/mg]
9	Storage Stability
99	Substrates and Products (Substrate)
23	Subunits
139	Synonyms
1	Systematic Name
9	Temperature Optimum [°C]
1	Temperature Range [°C]
5	Temperature Stability [°C]
30	Turnover Number [1/s]

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APPLICATION

ORGANISM

UNIPROT

COMMENTARY

LITERATURE

medicine

Homo sapiens

-

a C25U point mutation in the MTTV gene, gene encoding tRNAVal, is associated with a metabolic disorder resulting in the neonatal deaths of numerous siblings. In primary myoblasts and transmitochondrial cybrids established from the proband and offspring, the steady-state levels of the mutated mt-tRNAVal are greater than in the biopsy material, but are still lower than in control myoblasts. Decrease in steady-state mt-tRNAVal observed cell lines is caused by a rapid degradation of the deacylated form of the abnormal mt-tRNAVal. By both establishing the identity of the human mitochondrial valyltRNA synthetase then inducing its overexpression in transmitochondrial cell lines, steady-state levels of the mutated mt-tRNAVal can be partially restored

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