Data for: Tricarboxylic acid cycle enzyme activities in a mouse model of methylmalonic aciduria.

Here we examine the citric acid cycle enzymes activities and amounts for Cs, Idh2/3, Ogdh, Scs, Mmut, Sdh, Fh, Mdh2 from mitochondria isolated from model mouse model for methylmalonic acid (having a Mmut mutation (p.M698K) and MMut gene knockout, called here Mut-Ko/ki) and their heterozygote littermate (controls with wt/p.M698K). We also looked at a serial enzyme which we call MCM-SDH. A description of all techniques used for these assays is included in the paper (Wongkittichote et al).
Raw data from the enzyme assays is located in the excel spreadsheets attached (and labeled according to the enzyme assayed). The actual absorbance raw data as well as the labeling levels, averaging and standard deviations as well as initial graphs to calculate slope and so activity are included. Graph pad data include the protein quantification of immunoblots by measuring the amount of absorbance on a ChemiDoc™ MP Imaging System (170-8280; Bio-Rad) using Image Lab Software 5.1 by BioRad (Amountmutkoki). The enzyme calculations and graphing is also included via GraphPad as well statistics (mutenzymeactivity and Mutkokianalysis enzymeactivity).

本研究对甲基丙二酸酸（具有Mmut突变（p.M698K）及MMut基因敲除，简称Mut-Ko/ki）的模型小鼠及其同源杂合子（wt/p.M698K的正常对照组）的线粒体中柠檬酸循环酶的活性及含量进行了分析，包括Cs、Idh2/3、Ogdh、Scs、Mmut、Sdh、Fh、Mdh2等。此外，我们还考察了连续酶反应，称之为MCM-SDH。关于这些检测所使用技术的详细描述，可参见论文（Wongkittichote等）。酶活检测的原始数据位于所附的Excel电子表格中（并根据检测的酶进行标记）。其中包含了实际吸光度原始数据、标记水平、平均值及标准偏差，以及用于计算斜率和活性的初始图表。GraphPad数据包括通过ChemiDoc™ MP成像系统（170-8280；Bio-Rad）和BioRad的Image Lab软件5.1（Amountmutkoki）测量的免疫印迹蛋白定量，以及吸光度量。酶的计算和图表绘制也包含在GraphPad中，以及相关统计信息（mutenzymeactivity和Mutkokianalysis enzymeactivity）。