alanine + tRNA(Ala) + ATP => Ala-tRNA(Ala) + AMP + pyrophosphate

Stable Identifier
R-HSA-379864
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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AARS (cytosolic alanyl tRNA synthetase) catalyzes the reaction of alanine, tRNA(ala), and ATP to form Ala tRNA(Ala), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a monomer (Shiba et al. 1995). A mutation in the editing domain of the mouse Aars gene results in misincorporation of non-cognate amino acids into cellular proteins. This is associated with protein misfolding in Purkinje cells and a phenotype consistent with human ataxia (Lee et al. 2006).
Literature References
PubMed ID Title Journal Year
7654687 Human alanyl-tRNA synthetase: conservation in evolution of catalytic core and microhelix recognition

Schimmel, P, Suzuki, N, Ripmaster, T, Nichols, R, Shiba, K, Noda, T, Plotz, P

Biochemistry 1995
16906134 Editing-defective tRNA synthetase causes protein misfolding and neurodegeneration

Beebe, K, Schimmel, P, Sundberg, JP, Cook, SA, Nangle, LA, Davisson, MT, Ackerman, SL, Jang, J, Longo-Guess, CM, Lee, JW

Nature 2006
Participants
Participates
Catalyst Activity

alanine-tRNA ligase activity of AARS [cytosol]

Authored
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