Review Article

Neurodegeneration in Friedreich’s Ataxia: From Defective Frataxin to Oxidative Stress

Figure 1

Structures of the frataxin monomer and trimer, denoting the typical / fold in which the -helices pack against the -sheet strands. (a) Structure of the human frataxin monomer (PDB: 3S4M). (b) Structure of the yeast frataxin trimer (PDB: 3OEQ). Note that in the trimer structure, unwinding of the N-terminal -helix affords an interaction with a -sheet from the nearby subunit.
487534.fig.001a
(a)
487534.fig.001b
(b)