title: Human mitochondrial COX1 assembly into cytochrome c oxidase at a glance.
abstract: Mitochondria provide the main portion of cellular energy in form of ATP produced by the F1Fo ATP synthase, which uses the electrochemical gradient, generated by the mitochondrial respiratory chain (MRC). In human mitochondria, the MRC is composed of four multisubunit enzyme complexes, with the cytochrome c oxidase (COX, also known as complex IV) as the terminal enzyme. COX comprises 14 structural subunits, of nuclear or mitochondrial origin. Hence, mitochondria are faced with the predicament of organizing and controlling COX assembly with subunits that are synthesized by different translation machineries and that reach the inner membrane by alternative transport routes. An increasing number of COX assembly factors have been identified in recent years. Interestingly, mutations in several of these factors have been associated with human disorders leading to COX deficiency. Recently, studies have provided mechanistic insights into crosstalk between assembly intermediates, import processes and the synthesis of COX subunits in mitochondria, thus linking conceptually separated functions. This Cell Science at a Glance article and the accompanying poster will focus on COX assembly and discuss recent discoveries in the field, the molecular functions of known factors, as well as new players and control mechanisms. Furthermore, these findings will be discussed in the context of human COX-related disorders.
authors: Department of Cellular Biochemistry, University Medical Center Göttingen, 37073 Göttingen, Germany.
authors: Sven
authors: S
authors: Dennerlein
authors: Department of Cellular Biochemistry, University Medical Center Göttingen, 37073 Göttingen, Germany Max-Planck Institute for Biophysical Chemistry, 37077 Göttingen, Germany peter.rehling@medizin.uni-goettingen.de.
authors: Peter
authors: P
authors: Rehling
mesh_terms: D003576:Electron Transport Complex IV / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008928:Mitochondria / Q000201:enzymology* / Q000235:genetics; D024101:Mitochondrial Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: D024101:Mitochondrial Proteins; D003576:Electron Transport Complex IV
keywords: COX1; Complex IV; Cytochrome c oxidase; MITRAC; Mitochondria