Aminoacyl tRNA synthase complex-interacting multifunctional protein 2

AIMP2 (Aminoacyl tRNA synthase complex-interacting multifunctional protein 2) is a 35 kDa protein required for assembly and stability of the aminoacyl-tRNA synthase complex (UniProt: Q13155). It functions as a proapoptotic factor and regulates transcription through ubiquitination-mediated degradation of FUBP1, thereby suppressing MYC expression. AIMP2 also blocks MDM2-mediated degradation of p53, supporting its pro-apoptotic role.

In the nervous system, AIMP2 is associated with hypomyelinating leukodystrophy 17 (HLD17), an autosomal recessive neurodevelopmental disorder involving cerebral atrophy, cerebellar involvement, thin corpus callosum, and clinical features including seizures, spasticity, and developmental delay (UniProt: Q13155). These findings underscore its relevance to neuronal function and white matter integrity.

In Alzheimer's disease, AIMP2 is upregulated in post-mortem human AD brain tissue compared to age-matched controls, with a mean log2 fold-change of 0.31 across TMT-labeled proteomics analysis of subcellular fractions (Chaparral AD proteomics). This modest elevation suggests potential involvement in AD-associated pathology, possibly through altered apoptotic signaling or proteostatic mechanisms.