• GPI is a conserved post-translational modification essential for anchoring proteins to the cell surface; defects lead to inherited GPI deficiency (IGD).
• The review outlines the biosynthetic pathway of GPI-anchored proteins (GPI-APs) and summarizes clinical IGD cases from a molecular perspective.
• Current diagnostic and therapeutic approaches for IGD are reviewed, highlighting the need for improved management.
• Future research directions are discussed to enhance understanding and treatment of GPI-related disorders.