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3D schematic representation of complex II

Ribbon diagram (Richardson diagram) of complex II. The structure of the complex II (succinate dehydrogenase or succinate-ubiquinone oxidoreductase) bound to the inner phospholipid membrane of mitochondria. Complex II is a heterotetramer with 4 dissimilar subunits, 2 of which are hydrophilic (SdhA and SdhB), whereas the other 2 are hydrophobic membrane anchor subunits (SdhC and SdhD). The mitochondrial inter-membrane space is at the top of the image. Legend: SdhA is green, SdhB is teal, SdhC is fuchsia, and SdhD is yellowish green. Phospholipids surround the membrane subunits. Illustration by Richard Wheeler (Zephyris), 2006. (Courtesy of Wikimedia Commons. GNU Free Documentation License.)

Associated Disorders

  • Cardiomyopathy
  • Kearns-Sayre syndrome
  • Late-onset progressive neurodegeneration
  • Leigh disease
  • Seizures