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We identify L2HGDH as an integral component of the COQ metabolon, physically interacting with the complex and fueling coenzyme Q biosynthesis by reducing quinone intermediates.
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L-2-hydroxyglutarate recycling is linked to coenzyme Q biosynthesis
The mitochondrial COQ metabolon catalyzes the late stages of the biosynthesis of coenzyme Q, an essential and ubiquitous cofactor. Here, by integrating coevolution, coexpression, colocalization and do...