SRPRA (SRP receptor subunit alpha) is a critical component of the signal recognition particle (SRP) receptor complex that mediates cotranslational protein targeting to the endoplasmic reticulum (ER) 1. SRPRA functions by forming a GTP-dependent complex with the SRP54 subunit, driving SRP-mediated receptor compaction and GTPase rearrangement necessary for nascent secretory protein translocation across the ER membrane 2. This process ensures accurate targeting and processing of secretory proteins during translation. Dysfunction of SRPRA has significant clinical consequences. Biallelic genetic variants in SRPRA cause severe congenital neutropenia, characterized by global proteome aberrations affecting neutrophil granulocyte differentiation and protein homeostasis 3. Gene expression studies reveal SRPRA involvement in multiple stress-response pathways, including unfolded protein response, cellular stress responses, and apoptosis regulation 4. Additionally, SRPRA has emerged as a biomarker component in a lactylation-based prognostic signature for sepsis mortality prediction, suggesting its dysregulation reflects broader immune dysfunction during severe infection 5. These findings establish SRPRA as essential for both normal protein secretion machinery and maintaining hematopoietic cell homeostasis, with its dysfunction contributing to immunodeficiency and systemic inflammatory diseases.