ARV1 is a component of the enzyme initiating glycosylphosphatidylinositol biosynthesis.

ABSTRACT: Glycosylphosphatidylinositol (GPI) serves as a membrane anchor of numerous cell surface proteins. It is synthesized in the endoplasmic reticulum from phosphatidylinositol (PI) by stepwise reactions and transferred to the C terminus of the protein. Defects in genes involved in GPI biosynthesis affect the expression of GPI-anchored proteins or their structure, causing the neurological disorder, inherited GPI deficiency. Individuals with ARV1 deficiency have symptoms resembling inherited GPI deficiency, but how ARV1 regulates GPI biosynthesis is poorly understood. Here, we show that ARV1 acts as a component of the enzyme initiating GPI biosynthesis, GPI N-acetylglucosaminyltransferase (GPI-GnT) complex, which forms a ring structure as predicted by AlphaFold3. ARV1 associates with PIGQ, a GPI-

SUBMITTER: Lu T 

PROVIDER: S-EPMC12182360 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature