In this study, we analyzed the proteomic patterns of VO and ES of patients with MD and VS, which revealed the following: (1) Compared with the VS group, there were 148 DAPs in the MDES, and the DAPs were closely related to adhesion; (2) here were 119 DAPs in the MDVO, and the DAPs were closely related to synapse-related processes; (3) IL-1β increased glutamate levels by promoting GLS expression in HEI-OC1 cells, (4) GLS inhibitor CB-839 or the IL-1β receptor antagonist Anakinra alleviated the phenotype and reduced the glutamate levels in the EH mice. The gene discussed is GLS; the disease is Menkes disease.