Yubero Delia, Allen George, Artuch Rafael, Montero Raquel
Clinical Biochemistry and Molecular Medicine Department, Institut de Recerca Sant Joan de Déu and CIBERER-ISCIII, Passeig Sant Joan de Déu, 2, 08950 Esplugues, Barcelona, Spain.
Department of Blood Sciences, Royal Devon and Exeter NHS Foundation Trust, Exeter EX2 5DW, UK.
J Clin Med. 2017 Mar 24;6(4):37. doi: 10.3390/jcm6040037.
Coenzyme Q (CoQ) is a lipid that is ubiquitously synthesized in tissues and has a key role in mitochondrial oxidative phosphorylation. Its biochemical determination provides insight into the CoQ status of tissues and may detect CoQ deficiency that can result from either an inherited primary deficiency of CoQ metabolism or may be secondary to different genetic and environmental conditions. Rapid identification of CoQ deficiency can also allow potentially beneficial treatment to be initiated as early as possible. CoQ may be measured in different specimens, including plasma, blood mononuclear cells, platelets, urine, muscle, and cultured skin fibroblasts. Blood and urinary CoQ also have good utility for CoQ treatment monitoring.
辅酶Q（CoQ）是一种在组织中普遍合成的脂质，在线粒体氧化磷酸化中起关键作用。其生化测定有助于了解组织的辅酶Q状态，并可能检测到辅酶Q缺乏，这可能是由于辅酶Q代谢的遗传性原发性缺乏引起的，也可能继发于不同的遗传和环境条件。快速识别辅酶Q缺乏也可以尽早开始可能有益的治疗。辅酶Q可以在不同的标本中测量，包括血浆、血液单核细胞、血小板、尿液、肌肉和培养的皮肤成纤维细胞。血液和尿液中的辅酶Q在辅酶Q治疗监测中也有很好的用途。
