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. Author manuscript; available in PMC: 2014 Sep 30.
Published in final edited form as: Curr Top Membr. 2014;73:357–382. doi: 10.1016/B978-0-12-800223-0.00010-4

Figure 10.5.

Figure 10.5

The topology of human G6PT. The 429 amino acid residues are denoted by circles. The 34 amino acid residues altered by the 39 missense SLC37A4 mutations identified in GSD-Ib patients are shown in black. The signature motif (composed of amino acids 133–149) and the ER retention signal KKAE (composed of amino acids 426–429) are denoted by shaded circles. Three residues (p.Q133, p.A148, and p.G149) in the signature motif altered by missense SLC37A4 mutations identified in GSD-Ib patients are shown in dark shaded circles. The residues mutagenized for generating the glycosylation mutants p.T53N and p.S55N are denoted by arrows. Amino acids corresponding to essential residues in UhpT are indicated by arrowheads.