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. Author manuscript; available in PMC: 2011 Jul 1.
Published in final edited form as: Mech Ageing Dev. 2010 May 20;131(7-8):517–526. doi: 10.1016/j.mad.2010.05.003

Figure 1.

Figure 1

Mitochondrial abnormalities in SOD1-fALS.

The potential toxic effects of mutant SOD1 to mitochondria (indicated by black jagged symbols) are numerous. See also table 1 for a detailed list of reported mitochondrial abnormalities. Mitochondrial morphological abnormalities, including fragmentation and swelling due to expansion of the IMS, may involve interactions with proteins involved in mitochondrial fusion and fission, protein import, or Bcl proteins. Abnormalities of axonal transport of mitochondria along microtubule axes may result from mutant SOD1 interference with motors and cargo adaptors. Mutant SOD1 accumulation in mitochondria can affect availability of copper necessary for cuproenzymes. Mutant SOD1 in mitochondria can induce excess ROS and NO production. Mitochondrial mutant SOD1 can reduce association of cytochrome c with the IM, and enhance its release thereby activating caspase-dependent cell death. Mutant SOD1 reduces mitochondrial ATP synthesis, respiration, and electron transport chain (ETC) complex activities, especially complex IV. Decreased mitochondrial membrane potential can impair calcium uptake through the mitochondrial uniporter. Mutant SOD1 can causes sensitivity to mPTP opening resulting in release of ions and solutes.