Alpha- and gamma-adaptin-binding protein (AAGAB)

Alpha- and gamma-adaptin-binding protein (AAGAB) is a 34 kDa cytosolic protein found in most mammalian cells, characterized by two key regions: an N-terminal G-protein-like domain and a C-terminal dimerization domain (CTD). Its canonical function is as an assembly chaperone that facilitates the formation of AP-1 and AP-2 adaptor complexes, which are required for sorting proteins in clathrin-coated vesicle trafficking. AAGAB first exists as a homodimer; upon engaging AP-1 or AP-2 subunits (specifically the gamma subunit of AP-1, or the alpha subunit of AP-2), it dissociates into monomers to stabilize these subunits and enable complex assembly. Mutations in AAGAB, often leading to truncation and loss of the CTD, cause punctate palmoplantar keratoderma type I, a genetic skin disease characterized by hyperkeratotic lesions on palms and soles. AAGAB does not act as a classical therapeutic target (e.g., receptor, enzyme, transporter) and there are currently no known pharmacological agents or clinical biomarkers directly associated with its activity.

Other names

Alpha- and gamma-adaptin-binding protein p34AAGABFLJ11506p34KPPP1PPKP1PPKP1APunctate palmoplantar keratoderma type I