Phytanic acid impairs mitochondrial respiration through protonophoric action

Refsum disease is a rare, inherited neurodegenerative disorder characterized by accumulation of the dietary branched-chain fatty acid phytanic acid in plasma and tissues caused by a defect in the alphaoxidation pathway. The accumulation of phytanic acid is believed to be the main pathophysiological cause of the disease. However, the exact mechanism(s) by which phytanic acid exerts its toxicity have not been resolved. In this study, the effect of phytanic acid on mitochondrial respiration was investigated. The results show that in digitonin-permeabilized fibroblasts, phytanic acid decreases ATP synthesis, whereas substrate oxidation per se is not affected. Importantly, studies in intact fibroblasts revealed that phytanic acid decreases both the mitochondrial membrane potential and NAD(P)H autofluorescence. Taken together, the results described here show that unesterified phytanic acid exerts its toxic effect mainly through its protonophoric action, at least in human skin fibroblasts. Received 4 August 2007; received after revision 26 September 2007; accepted 10 October 2007 J. C. Komen, F. Distelmaier: These authors contributed equally to this work.     

Intelligent Control Mechanism of Part Picking Operations of Automated Warehouse

This paper studies the part picking operations of a ut omated warehouse. It assumed the demand of picking orders of automated warehouse are dynamic generated. Once the picking orders of certain period of time are kn own, it is necessary to decide an efficient order picking sequence and routing t o minimize the total travel distance to complete those orders. Assumed there are n i items to be picked in order O i. Each item in the picking ord er is located in different locations in the warehouse. Since i...     

新型丙烯酸多元共聚物增稠性的研究

1 实验部分1.1 原料及其规格(见表1)1.2 乳胶液的合成方法 在装有搅拌器、回流冷凝管、温度计和滴液漏斗的250ml三瓶中进行乳液共聚合,其反应温度为85±1℃。不同聚合方法和加料顺序如下。1.2.1 半连续加料法 乳化剂和水溶解均一后,升至一定温度,加入全部引发剂;继续升温,然后滴加全部AA和部分BA及MMA、St混合单体混合物;全部加完后再将剩余混合单体在1h内滴加完,保温一定时间,降温至40℃出料。     

纳米固体超强酸SO42-/ZnO催化合成乙酸异戊酯的研究

本文利用纳米固体超强酸SO4^2-/ZnO催化合成乙酸异戊酯的最佳反应条件,既反应时间2.0h,催化剂用量为酸质量的1.0%,酸醇比为1∶2,验证实验产率为96.7%,且该实验反应时间短,无腐蚀无污染,催化剂可回收可重复利用.     

The Three-component One-pot Synthesis of 4,6-Diarylpyrimidin-2(1h)-ones and 9-Phenyl-8-oxa-10,12-diaza-tricyclo[7.3.1.0~(2,7)]trideca-2(7),3,5-trien-11-one

1 Results Pyrimidinones (PMs) are a class of important heterocycles which have been well documented throughout the literature due to their biological importance. They exhibit a wide range of pharmaceutical and therapeutic properties[1].A rapid and efficient one-pot method for the synthesis of 4,6-diarylpyrimidin-2(1H)- ones and related heterocycles is described.The condensation of acetophenone derivatives,aldehydes and urea in the presence of sulfamic acid was employed to synthesize a variety of pyrimid...     

制备介孔复合环境材料的酸洗刻蚀工艺

探索了一种制备介孔复合环境材料的方法--破坏式造孔与有机复合相结合,使最终产品可大量吸附污染水体中的各种污染物并具有一定的杀菌功能.该方法通过酸洗刻蚀,达到了去除原材料中的杂质和刻蚀孔径的目的,所制备的中间产品24h吸湿率为原材料的2倍,染料吸附量为原材料的1.6倍.     

两步法磷酸生产余热回收改造及热经济学分析

中国磷酸生产过程中的热浪费和热污染问题非常严重。解决这一问题将带来巨大的经济效益和社会效益。该文分别从能和可用能两个角度对一种新的燃烧水合两步法制取磷酸系统的余热回收改造方案进行分析,得到了改造前后整个系统的能流分布和可用能流分布,以及各个环节的能效率和可用能效率,并使用热经济学分析的方法,结合环境政策和资源占有对改造前后的多元产品系统进行可用能成本折算和分析。结果表明这种余热回收改造方案在经济效益、环境保护和节约资源3个方面具有明显的优越性。     

通过实测室温确定住宅的自然通风状况

住宅自然通风状况的研究有助于住宅设计,使之更加有利于改善空气品质和节能。通过分析比较实测室温和模拟室温,根据状态空间法逐时确定出房间的通风换气频率。并以北京市的3户住宅为研究对象,从以下几个方面讨论计算结果的有效性,模拟室温的计算误差满足精度要求;计算通风换气频率一般小于10h-1,但最高可达50h-1,同时,计算通风换气频率的变化表现出了风压和住户调节行为的影响。故计算通风换气频率大体上符合住宅实际的自然通风状况。     

乳化液膜法净化湿法磷酸除镁研究

采用乳化液膜技术对湿法磷酸进行脱镁净化是一种净化湿法磷酸的新途径。采用正交试验设计方法组织实验，结果表明在实验研究范围内，萃取剂对湿法磷酸中脱镁效果有高度显著的影响，而表面活性剂、膜试剂和反萃取剂对镁的脱除有一定影响。