Secondary coenzyme Q deficiency in neurological disorders

Coenzyme Q (CoQ) is a ubiquitous lipid serving essential cellular functions. It the only component of mitochondrial respiratory chain that can be exogenously absorbed. Here, we provide an overview current knowledge, controversies, and open questions about CoQ intracellular tissue distribution, in particular brain skeletal muscle. We discuss human neurological diseases mouse models associated with secondary deficiency these tissues highlight pharmacokinetic anatomical challenges exogenous biodistribution, recent improvements formulations imaging, as well alternative therapeutical strategies to supplementation. The last section proposes possible mechanisms underlying emphasis on neuromuscular disorders.