AGBL1 (AGBL carboxypeptidase 1) is a metallocarboxypeptidase that mediates deglutamylation of tubulin and non-tubulin proteins by removing polyglutamate side chains from the gamma-carboxyl group of glutamate residues 1. The protein localizes primarily to the cytoplasm and plays a role in controlling the length of polyglutamate side chains 12. AGBL1 has been identified as expressed in corneal endothelium through serial analysis of gene expression and immunohistochemical staining 1. The protein interacts biochemically with TCF4, a transcription factor associated with Fuchs corneal dystrophy (FCD) 1. Mutations in AGBL1 have been reported in association with late-onset Fuchs corneal dystrophy, including nonsense and missense variants that alter protein localization and diminish interaction with TCF4 1. However, recent systematic reviews challenge AGBL1's causal role in FCD, noting lack of expression in corneal endothelium, conflicting functional evidence, and absence of pathogenic variants meeting ACMG criteria 34. Additionally, AGBL1 variants have been associated with leukocyte telomere length in cattle and lung cancer risk in certain populations, suggesting broader cellular functions 52.