Editing Coenzyme Q10 (section)

==Biosynthesis==
Biosynthesis occurs in most human tissue. There are three major steps:
# Creation of the [[benzoquinone]] structure (using [[phenylalanine]] or [[tyrosine]], via [[4-hydroxybenzoate]])
# Creation of the [[isoprene]] side chain (using [[acetyl-CoA]])
# The joining or [[condensation reaction|condensation]] of the above two structures

The initial two reactions occur in [[mitochondria]], the [[endoplasmic reticulum]], and [[peroxisome]]s, indicating multiple sites of synthesis in animal cells.<ref name="pmid20494114">{{cite journal | vauthors = Bentinger M, Tekle M, Dallner G | title = Coenzyme Q--biosynthesis and functions | journal = Biochemical and Biophysical Research Communications | volume = 396 | issue = 1 | pages = 74–79 | date = May 2010 | pmid = 20494114 | doi = 10.1016/j.bbrc.2010.02.147 }}</ref>

An important enzyme in this pathway is [[HMG-CoA reductase]], usually a target for intervention in cardiovascular complications. The "statin" family of cholesterol-reducing medications inhibits HMG-CoA reductase. One possible side effect of statins is decreased production of CoQ<sub>10</sub>, which may be connected to the development of [[myopathy]] and [[rhabdomyolysis]]. However, the role statins play in CoQ deficiency is controversial. Although statins reduce blood levels of CoQ, studies on the effects of muscle levels of CoQ are yet to come. CoQ supplementation also does not reduce side effects of statin medications.<ref name="pmid21844807"/><ref name="pmid28546301"/>

Genes involved include ''[[PDSS1]]'', ''[[PDSS2]]'', ''[[COQ2]]'', and ''[[ADCK3]]'' (''COQ8'', ''CABC1'').<ref name="Espinós-2009">{{Cite book | vauthors = Espinós C, Felipo V, Palau F |url=https://books.google.com/books?id=uxQ_pjKNhE8C&pg=PA122 |title=Inherited Neuromuscular Diseases: Translation from Pathomechanisms to Therapies |date=2009 |publisher=Springer |isbn=978-90-481-2812-9 |pages=122ff |access-date=4 January 2011}}</ref>

Organisms other than humans produce the benzoquinone and isoprene structures from somewhat different source chemicals. For example, the bacteria ''[[E. coli]]'' produces the former from [[chorismate]] and the latter from a non-[[mevalonate]] source. The common yeast ''[[S. cerevisiae]]'', however, derives the former from either chorismate or tyrosine and the latter from [[mevalonate]]. Most organisms share the common 4-hydroxybenzoate intermediate, yet again uses different steps to arrive at the "Q" structure.<ref name="pmid11583838">{{cite journal | vauthors = Meganathan R | title = Ubiquinone biosynthesis in microorganisms | journal = FEMS Microbiology Letters | volume = 203 | issue = 2 | pages = 131–139 | date = September 2001 | pmid = 11583838 | doi = 10.1111/j.1574-6968.2001.tb10831.x | doi-access = free }}</ref>