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Target intelligence / Profile preview
Post-GPI attachment to proteins factor 2 (PGAP2)
- Target
- PGAP2
- Molecular classification
- Enzyme (specifically, participates in lipid remodeling of protein anchors), Golgi/ER-resident membrane protein, Other (cannot be classified into canonical receptor or ion channel families)
01
Overview
Post-GPI attachment to proteins factor 2 (PGAP2) is a transmembrane protein localized in the Golgi apparatus and endoplasmic reticulum (ER). It performs a key enzymatic role in one of the final steps of glycosylphosphatidylinositol (GPI) anchor biosynthesis, specifically catalyzing the addition of a saturated fatty acid (stearic acid) to the lipid segment of the GPI anchor. This lipid remodeling is essential for GPI-anchored proteins to associate properly in lipid rafts at the plasma membrane, influencing cell signaling and protein localization. PGAP2 deficiency results in the abnormal processing of GPI-APs, leading to altered cell-surface protein expression that underpins rare congenital glycosylation disorders with neurodevelopmental involvement and elevated alkaline phosphatase. PGAP2 has homologs in other eukaryotes (e.g., yeast Cwh43p), where it is involved in cell wall biogenesis. PGAP2 is not a direct pharmacological target and no drugs are known to interact with it[1][2][3][4].
- Other names
- PGAP2Acyltransferase PGAP2FRAG1CWH43-NFGF receptor-activating protein 1Post-GPI attachment to proteins factor 2cell wall biogenesis 43 N-terminal homolog (S. cerevisiae)HPMRS3MRT17MRT21
02
Biological functions
GPI anchor biosynthesis and remodelingFacilitates cell-surface expression of glycosylphosphatidylinositol-anchored proteins (GPI-APs)Modifies the lipid component of GPI anchors, critical for localization and clustering in membrane domains (lipid rafts), which impact signaling and cell surface architecture
03
Disease associations
Congenital disorders of glycosylation, specifically PGAP2-CDG (CDG for PGAP2)Neurological disorders, including intellectual disability, epilepsy, dysmorphisms, and elevated alkaline phosphataseCell wall defects in yeast (homologous protein, Cwh43p), linking to cell wall biogenesis research
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