Editing Coenzyme Q10 (section)

==Deficiency==
There are two major pathways of deficiency of CoQ<sub>10</sub> in humans: reduced [[biosynthesis]], and increased use by the body.<ref name="pmid25091424">{{cite journal |vauthors=Desbats MA, Lunardi G, Doimo M, Trevisson E, Salviati L |title=Genetic bases and clinical manifestations of coenzyme Q10 (CoQ 10) deficiency |journal=J Inherit Metab Dis |volume=38 |issue=1 |pages=145–56 |date=January 2015 |pmid=25091424 |doi=10.1007/s10545-014-9749-9 |url=}}</ref> Biosynthesis is the major source of CoQ<sub>10</sub>. Biosynthesis requires at least 15 [[gene]]s, and mutations in any of them can cause CoQ deficiency.<ref name="pmid25091424"/> CoQ<sub>10</sub> levels also may be affected by other genetic defects (such as mutations of [[mitochondrial DNA]], ''[[ETFDH]]'', ''[[APTX]]'', ''[[FXN]]'', and ''[[BRAF (gene)|BRAF]]'', genes that are not directly related to the CoQ<sub>10</sub> biosynthetic process).<ref name="pmid25091424"/> Some of these, such as mutations in ''[[COQ6]]'', can lead to serious diseases such as steroid-resistant [[nephrotic syndrome]] with sensorineural [[deafness]].<ref>{{cite journal|doi=10.1172/JCI45693 |title=COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness |date=2011 |journal=Journal of Clinical Investigation |volume=121 |issue=5 |pages=2013–2024 |pmid=21540551 |pmc=3083770 |vauthors = Heeringa SF, Chernin G, Chaki M, Zhou W, Sloan AJ, Ji Z, Xie LX, Salviati L, Hurd TW, Vega-Warner V, Killen PD, Raphael Y, Ashraf S, Ovunc B, Schoeb DS, McLaughlin HM, Airik R, Vlangos CN, Gbadegesin R, Hinkes B, Saisawat P, Trevisson E, Doimo M, Casarin A, Pertegato V, Giorgi G, Prokisch H, Rötig A, Nürnberg G, Becker C }}</ref><ref>{{cite journal|doi=10.1002/jmd2.12068 |title=''COQ6'' mutation in patients with nephrotic syndrome, sensorineural deafness, and optic atrophy |date=2020 |journal=Jimd Reports |volume=54 |issue=1 |pages=37–44 |pmid=32685349 |pmc=7358665 | vauthors = Justine Perrin R, Rousset-Rouvière C, Garaix F, Cano A, Conrath J, Boyer O, Tsimaratos M }}</ref><ref>{{cite web | url=https://www.ncbi.nlm.nih.gov/medgen/886260 | title=Nephrotic Syndrome - COQ6 Associated (Concept Id: C4054393) - MedGen - NCBI | access-date=6 April 2024 | archive-date=6 April 2024 | archive-url=https://web.archive.org/web/20240406102811/https://www.ncbi.nlm.nih.gov/medgen/886260 | url-status=live }}</ref>

===Assessment===
Although CoQ<sub>10</sub> may be measured in [[blood plasma]], these measurements reflect dietary intake rather than tissue status. Currently, most clinical centers measure CoQ<sub>10</sub> levels in cultured skin [[fibroblast]]s, muscle [[biopsy|biopsies]], and blood mononuclear cells.<ref name="pmid21844807">{{cite journal | vauthors = Trevisson E, DiMauro S, Navas P, Salviati L | title = Coenzyme Q deficiency in muscle | journal = Current Opinion in Neurology | volume = 24 | issue = 5 | pages = 449–456 | date = October 2011 | pmid = 21844807 | doi = 10.1097/WCO.0b013e32834ab528 | hdl-access = free | hdl = 10261/129020 }}</ref> Culture fibroblasts can be used also to evaluate the rate of endogenous CoQ<sub>10</sub> biosynthesis, by measuring the uptake of [[carbon-14|<sup>14</sup>C]]-[[isotopic labeling|labeled]] [[p-Hydroxybenzoic acid|''p''-hydroxybenzoate]].<ref name="pmid18387363">{{cite journal | vauthors = Montero R, Sánchez-Alcázar JA, Briones P, Hernández AR, Cordero MD, Trevisson E, Salviati L, Pineda M, García-Cazorla A, Navas P, Artuch R | title = Analysis of coenzyme Q10 in muscle and fibroblasts for the diagnosis of CoQ<sub>10</sub> deficiency syndromes | journal = Clinical Biochemistry | volume = 41 | issue = 9 | pages = 697–700 | date = June 2008 | pmid = 18387363 | doi = 10.1016/j.clinbiochem.2008.03.007 | hdl-access = free | hdl = 11577/2447079 }}</ref>

CoQ<sub>10</sub> is studied as an adjunctive therapy to reduce inflammation in [[periodontitis]].<ref name="pmid37592831">{{cite journal |vauthors=Fawzy El-Sayed KM, Cosgarea R, Sculean A, Doerfer C |title=Can vitamins improve periodontal wound healing/regeneration? |journal=Periodontol 2000 |volume=94 |issue=1 |pages=539–602 |date=February 2024 |pmid=37592831 |doi=10.1111/prd.12513 |url=|doi-access=free }}</ref>

===Statins===
Although statins may reduce CoQ<sub>10</sub> in the blood it is unclear if they reduce CoQ<sub>10</sub> in muscle.<ref name="pmid28546301"/> Evidence does not support that supplementation improves statin side effects.<ref name="pmid28546301">{{cite journal | vauthors = Tan JT, Barry AR | title = Coenzyme Q10 supplementation in the management of statin-associated myalgia | journal = American Journal of Health-System Pharmacy | volume = 74 | issue = 11 | pages = 786–793 | date = June 2017 | pmid = 28546301 | doi = 10.2146/ajhp160714 | s2cid = 3825396 | doi-access = free }}</ref><ref>{{Cite journal |last1=Kennedy |first1=Cormac |last2=Köller |first2=Yasmin |last3=Surkova |first3=Elena |date=2020-04-01 |title=Effect of Coenzyme Q10 on statin-associated myalgia and adherence to statin therapy: A systematic review and meta-analysis |journal=Atherosclerosis |language=en |volume=299 |pages=1–8 |doi=10.1016/j.atherosclerosis.2020.03.006 |pmid=32179207 |doi-access=free }}</ref>