Plasma Coenzyme Q10 Levels in Patients With Multiple System Atrophy

• DOI: 10.1001/jamaneurol.2016.1325
• Publication Date: 2016-06-30T04:33:11Z
• Authors: Jun Mitsui; Takashi Matsukawa; Tsutomu Yasuda; Hiroyuki Ishiura; Shoji Tsuji
• Keywords: Coenzyme Q10; Pathogenesis

<h3>Importance</h3> Multiple system atrophy (MSA) is an intractable neurodegenerative disease characterized by autonomic failure in addition to various combinations of parkinsonism, cerebellar ataxia, and pyramidal dysfunction. It has recently been reported that functionally impaired variants of<i>COQ2</i>, which encodes essential enzyme the biosynthetic pathway coenzyme Q10 (CoQ10), are associated with MSA. However, little known about role CoQ10 pathogenesis <h3>Objective</h3> To compare levels plasma patients MSA those age-, sex-, and<i>COQ2</i>genotype–matched controls. <h3>Design, Setting, Participants</h3> We enrolled 44 Japanese 39 controls from September 1, 2012, December 31, 2015. Patients were diagnosed on basis second consensus criteria at least 2 neurologists. Plasma measured high-performance liquid chromatography electrochemical detection. Sanger sequencing of<i>COQ2</i>was performed determine the<i>COQ2</i>genotypes. logistic regression analysis was association between level. <h3>Main Outcomes Measures</h3> compared after adjusting for age, sex, and<i>COQ2</i>genotype. <h3>Results</h3> Among (mean [SD] 63.7 [8.3] years) 60.3 [13.0] years), mean (SD) level lower than (0.51 [0.22] vs 0.72 [0.42] µg/mL;<i>P</i> = .01) (difference medians: −0.14; 95% CI, –0.25 –0.03). The variant parkinsonian 0.58 (0.19) 0.49 (0.26) µg/mL, respectively. After and<i>COQ2</i>genotype, significantly (95% 0.10; range, 0.02 0.66) (<i>P</i> .02). <h3>Conclusions Relevance</h3> Our data showed decreased regardless the<i>COQ2</i>genotype, supporting a hypothesis supplementation beneficial