Fig. 5: Locations of PFBC disease mutations mapped on the intracellular gate open (iO) structure of XPR1.
From: Transport and InsP8 gating mechanisms of the human inorganic phosphate exporter XPR1

Residues of which missense mutations are known to cause neurological disease phenotypes are represented as sticks. Nitrogen, blue; oxygen, red. The residues are clustered near protein regions critical for function, including (1) the substrate translocation pathway, (2) the Arg extension and capture pocket, and (3) the intracellular gate or C-plug.