Figure 5 | Scientific Reports

Figure 5

From: Identification of G8969>A in mitochondrial ATP6 gene that severely compromises ATP synthase function in a patient with IgA nephropathy

Figure 5

Topological location of the aS148 residue of a-subunit of human ATP synthase in the inner mitochondrial membrane.

(A) Alignment of a-subunits from mitochondrial and bacterial origins. The G8969>A mutation leads to the replacement with asparagine of the evolutionary conserved serine at position 148 of the human a-subunit. (B,C) Structural homology model of human c8-ring (PDB # 2XND) in complex with four helical segments of the a-subunit (aMH2-5), coloured from blue (N-Term) to red (C-Term) viewed from the matrix (B) and from the membrane plane (C). The side chain of aS148 residue is shown as sticks, the cE58 and aR159 are as spheres. The arrowed line indicates the pathway for protons during ATP synthesis. Protons enter and exit the c-ring/a-subunit through hydrophilic clefts in the membrane represented by the grey areas surrounded by dashed lines31.

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