Dr. Frederick Crane was a researcher under Dr. David E. Green in the mid-1950s, during the early days of the University of Wisconsin-Madison (UW-Madison) Enzyme Institute, when he made his defining discovery. The lab group was on a mission to determine, bit by bit, how mitochondria -- the power plants of cells -- generate the energy required to sustain life. What Dr. Crane found, a compound called coenzyme Q, was a missing piece of the puzzle and became a major part of the legacy of mitochondrial research at UW-Madison. But it was no accident. "It was the result of a long train of investigation into a mechanism of, and compounds involved in, biological energy conversion," Dr. Crane wrote in a 2007 review article of his discovery. Almost six decades later, that "long train" has grown even longer. Dr. Dave Pagliarini, a UW-Madison assistant professor of biochemistry, has established a new laboratory studying these dynamic organelles, the mitochondria. He recently published two studies shedding more light on coenzyme Q and how it's made, one in PNAS online on October 22, 2014. and another on December 11, 2014 in Molecular Cell. "Mitochondria are tiny structures in nearly all of our cells that are essential for producing our cellular energy and that house a wide array of metabolic processes," Dr. Pagliarini says. "When mitochondria don't work properly, many different human diseases can arise." These include cerebellar ataxia, certain kidney diseases, and severe childhood-onset multisystemic diseases. Coenzyme Q deficiency is a hallmark of these diseases, but scientists aren't sure why. "Nearly 60 years later, there is still much we don't know about how mitochondria make coenzyme Q and that has complicated our ability to target this pathway therapeutically," Dr. Pagliarini says.
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