Phosphatidylinositol N-acetylglucosaminyltransferase subunit H (PIGH)

Phosphatidylinositol N-acetylglucosaminyltransferase subunit H (PIGH) is an endoplasmic reticulum-associated enzyme component required for the biosynthesis of glycosylphosphatidylinositol (GPI) anchors[1][3][7][8][9]. This subunit is part of the GPI N-acetylglucosaminyl (GlcNAc) transferase complex, which catalyzes the transfer of N-acetylglucosamine (GlcNAc) to phosphatidylinositol (PI), the first step in GPI anchor biosynthesis[1][3][7][8][9]. GPI anchors tether a wide range of proteins—critical for neuronal development, embryogenesis, and immune signaling—to the cell surface. Genetic variants or silencing of PIGH impair GPI anchor formation and surface display of GPI-anchored proteins, leading to neurological, developmental, and immune dysfunction, as well as resistance to some targeted therapies in hematologic malignancies[1][3][7].

Other names

Phosphatidylinositol glycan anchor biosynthesis class HPIGHGPI-HPhosphatidylinositol-glycan biosynthesis class H protein