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

Stable Identifier
R-HSA-379864
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
16906134 Editing-defective tRNA synthetase causes protein misfolding and neurodegeneration

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

Nature 2006
7654687 Human alanyl-tRNA synthetase: conservation in evolution of catalytic core and microhelix recognition

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

Biochemistry 1995
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
alanine-tRNA ligase activity of AARS [cytosol]
Physical Entity
Activity
Authored
Reviewed
Created