Singh Inderjit, Paintlia Ajaib S, Khan Mushfiquddin, Stanislaus Romesh, Paintlia Manjeet K, Haq Ehtishamul, Singh Avtar K, Contreras Miguel A
Department of Pediatrics, Medical University of South Carolina, 171 Ashley Avenue, Charleston SC 29425, USA.
Brain Res. 2004 Oct 1;1022(1-2):1-11. doi: 10.1016/j.brainres.2004.06.059.
Peroxisomes are ubiquitous subcellular organelles and abnormality in their biogenesis and specific gene defects leads to fatal demyelinating disorders. We report that neuroinflammatory disease in brain of experimental autoimmune encephalomyelitis (EAE) rats decreased the peroxisomal functions. Degradation of very long chain fatty acids decreased by 47% and resulted in its accumulation (C26:0, 40%). Decreased activity (66% of control) of dihydroxyacetonephosphate acyltransferase (DHAP-AT), first enzyme in plasmalogens biosynthesis, resulted in decreased levels of plasmalogens (16-30%). Catalase activity, a peroxisomal enzyme, was also reduced (37%). Gene microarray analysis of EAE spinal cord showed significant decrease in transcripts encoding peroxisomal proteins including catalase (folds 3.2; p<0.001) and DHAP-AT (folds 2.6; p<0.001). These changes were confirmed by quantitative reverse transcription polymerase chain reaction (RT-PCR) analysis, suggesting that decrease of peroxisomal functions in the central nervous system will have negative consequences for myelin integrity and repair because these lipids are major constituents of myelin. However, lovastatin (a cholesterol lowering and anti-inflammatory drug) administered during EAE induction provided protection against loss/down-regulation of peroxisomal functions. Attenuation of induction of neuroinflammatory mediators by statins in cultured brain cells [J. Clin. Invest. 100 (1997) 2671-2679], and in central nervous system of EAE animals and thus the EAE disease [J. Neurosci. Res. 66 (2001) 155-162] and the studies described here indicate that inflammatory mediators have a marked negative effect on peroxisomal functions and thus on myelin assembly and that these effects can be prevented by treatment with statins. These observations are of importance because statins are presently being tested as therapeutic agents against a number of neuroinflammatory demyelinating diseases.
过氧化物酶体是普遍存在的亚细胞器,其生物发生异常和特定基因缺陷会导致致命的脱髓鞘疾病。我们报告,实验性自身免疫性脑脊髓炎(EAE)大鼠脑内的神经炎症性疾病会降低过氧化物酶体功能。极长链脂肪酸的降解减少了47%,导致其积累(C26:0,40%)。二羟基丙酮磷酸酰基转移酶(DHAP-AT)是缩醛磷脂生物合成中的首个酶,其活性降低(为对照的66%),导致缩醛磷脂水平降低(16 - 30%)。过氧化氢酶活性作为一种过氧化物酶体酶也降低了(37%)。EAE脊髓的基因微阵列分析显示,编码包括过氧化氢酶(3.2倍;p<0.001)和DHAP-AT(2.6倍;p<0.001)在内的过氧化物酶体蛋白的转录本显著减少。这些变化通过定量逆转录聚合酶链反应(RT-PCR)分析得到证实,表明中枢神经系统中过氧化物酶体功能的降低将对髓鞘完整性和修复产生负面影响,因为这些脂质是髓鞘的主要成分。然而,在EAE诱导期间给予洛伐他汀(一种降低胆固醇和抗炎药物)可防止过氧化物酶体功能的丧失/下调。他汀类药物在培养的脑细胞中对神经炎症介质诱导的减弱作用[《临床研究杂志》100 (1997) 2671 - 2679],以及在EAE动物中枢神经系统中对神经炎症介质诱导的减弱作用,进而对EAE疾病的减弱作用[《神经科学研究》66 (2001) 155 - 162],以及此处描述的研究表明,炎症介质对过氧化物酶体功能以及髓鞘组装具有显著的负面影响,并且这些影响可以通过他汀类药物治疗来预防。这些观察结果具有重要意义,因为目前正在测试他汀类药物作为针对多种神经炎症性脱髓鞘疾病的治疗药物。