SCYE1 iso2: Non-catalytic component of the multisynthase complex. Stimulates the catalytic activity of cytoplasmic arginyl-tRNA synthase. Binds tRNA. Possesses inflammatory cytokine activity. Negatively regulates TGF-beta signaling through stabilization of SMURF2 by binding to SMURF2 and inhibiting its SMAD7-mediated degradation. Involved in glucose homeostasis through induction of glucagon secretion at low glucose levels. Promotes dermal fibroblast proliferation and wound repair. Regulates KDELR1- mediated retention of HSP90B1/gp96 in the endoplasmic reticulum. Plays a role in angiogenesis by inducing endothelial cell migration at low concentrations and endothelian cell apoptosis at high concentrations. Induces maturation of dendritic cells and monocyte cell adhesion. Modulates endothelial cell responses by degrading HIF-1A through interaction with PSMA7. Defects in AIMP1 are the cause of leukodystrophy hypomyelinating type 3 (HLD3). A severe autosomal recessive hypomyelinating leukodystrophy characterized by early infantile onset of global developmental delay, lack of development, lack of speech acquisition, and peripheral spasticity associated with decreased myelination in the central nervous system. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Motility/polarity/chemotaxis
Chromosomal Location of Human Ortholog: 4q24
Cellular Component: Golgi apparatus; extracellular space; aminoacyl-tRNA synthetase multienzyme complex; transport vesicle; cell surface; membrane; endoplasmic reticulum; cytoplasm; cytosol; nucleus
Molecular Function: methionine-tRNA ligase activity; protein binding; protein homodimerization activity; cytokine activity; tRNA binding
Biological Process: tRNA aminoacylation for protein translation; cell-cell signaling; apoptosis; negative regulation of endothelial cell proliferation; methionyl-tRNA aminoacylation; response to wounding; glucose metabolic process; gene expression; angiogenesis; cell adhesion; signal transduction; inflammatory response; chemotaxis; leukocyte migration
Disease: Leukodystrophy, Hypomyelinating, 3
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