EEF1AKMT1 (also known as N6AMT2) is a protein lysine methyltransferase that selectively catalyzes trimethylation of elongation factor 1A (eEF1A) at lysine 79 1. This enzyme is conserved from yeast to humans, with the trimethylation of Lys(79) in eEF1A preserved across eukaryotes, suggesting functional importance 1. EEF1A is an essential, highly methylated protein that facilitates translational elongation by delivering aminoacyl-tRNAs to ribosomes 1. EEF1AKMT1 represents one of multiple independent methyltransferases that modify eEF1A, indicating extensive regulation of translation elongation by posttranslational modification 1. In disease contexts, deletion of EEF1AKMT1 located on chromosome 13.11 has been associated with a novel syndromic progressive corneal opacification featuring epithelial and stromal changes, alongside sensorineural hearing loss and tracheal/laryngeal abnormalities in affected families 2. Pathway analysis of tissues from affected individuals revealed upregulation of collagen metabolism and extracellular matrix formation, suggesting that loss of EEF1AKMT1 function may dysregulate ECM homeostasis 2. These findings highlight EEF1AKMT1's role in both basic translational regulation and human genetic disease.