Literature summary for 6.1.1.4 extracted from

Perli, E.; Giordano, C.; Pisano, A.; Montanari, A.; Campese, A.F.; Reyes, A.; Ghezzi, D.; Nasca, A.; Tuppen, H.A.; Orlandi, M.; Di Micco, P.; Poser, E.; Taylor, R.W.; Colotti, G.; Francisci, S.; Morea, V.; Frontali, L.; Zeviani, M.; dAmati, G.
The isolated carboxy-terminal domain of human mitochondrial leucyl-tRNA synthetase rescues the pathological phenotype of mitochondrial tRNA mutations in human cells (2014), EMBO Mol. Med., 6, 169-182.

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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-leucine + tRNALeu	Homo sapiens	-	AMP + diphosphate + L-leucyl-tRNALeu	-	?

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	-	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
ATP + L-leucine + tRNALeu	-	Homo sapiens	AMP + diphosphate + L-leucyl-tRNALeu	-	?

Synonyms

Synonyms	Comment	Organism
Leucyl-tRNA synthetase	-	Homo sapiens
LeuRS	-	Homo sapiens

Cofactor

Cofactor	Comment	Organism	Structure
ATP	-	Homo sapiens

General Information

General Information	Comment	Organism
physiological function	the carboxy-terminal domain of human mitochondrial leucyl-tRNA synthetase can be used to correct mitochondrial dysfunctions caused by mitochondrial tRNA mutations like the phenotype of m.3243A>G MTTL1 mutant cybrids	Homo sapiens

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Release 2023.1 (January 2023)