The protein mutated in Friedrich's Ataxia, frataxin, localizes to this organelle.
The protein mutated in Friedrich's Ataxia, frataxin, localizes to this organelle. This organelle is stained by a series of rosamine derivatives sold by Invitrogen, named [this organelle]-"tracker". Antibodies to proteins within it are present in 95% of primary billiary cirrhosis cases. Another dye that localizes here is nonyl acridine orange, which stains a bicyclic phospholipid called cardiolipin. The glycerol phosphate and malate-aspartate shuttles move reducing equivalents into it, and with the aid of the (*) carnitine shuttle it carries out beta-oxidation.  MELAS and ragged red fiber disease are caused by defects in it, as is Leber's Neuropathy. Its contains NADH dehydrogenase, succinate dehydrogenase, and ubiquinone, all components of the electron transport chain. Also containing ATP synthase and Krebs cycle enzymes, for 10 points, name this double-membraned organelle with a cristae and matrix.
The protein mutated in Friedrich's Ataxia, frataxin, localizes to this organelle.
{Mitochondria}