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首页> 外文期刊>Cell biology international. >Mitochondrial targeting of human protoporphyrinogen oxidase.
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Mitochondrial targeting of human protoporphyrinogen oxidase.

机译：线粒体靶向人原卟啉原氧化酶。

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Variegate porphyria is an autosomal dominant disorder of heme metabolism resulting from a deficiency in protoporphyrinogen oxidase, an enzyme located on the inner mitochondrial membrane. This study examined the effect of three South African VP-causing mutations (H20P, R59W, R168C) on mitochondrial targeting. Only H20P did not target, and of eight protoporphyrinogen oxidase-GFP chimeric fusion proteins created, N-terminal residues 1-17 were found to be the minimal protoporphyrinogen oxidase sequence required for efficient mitochondrial targeting. Removal of this N-terminal sequence displayed mitochondrial localization, suggesting internal mitochondrial targeting signals. In addition, six constructs were engineered to assess the effect of charge and helicity on mitochondrial targeting of the protein. Of those engineered, only the PPOX20/H20P-GFP construct abolished mitochondrial targeting, presumably through disruption of the protoporphyrinogen oxidase alpha-helix. Based on our results we propose a mechanism for protoporphyrinogen oxidase targeting to the mitochondrion.
机译：Variegate卟啉病是原卟啉原氧化酶（一种位于线粒体内膜上的酶）缺乏导致的血红素代谢常染色体显性疾病。这项研究检查了三个南非VP致突变（H20P，R59W，R168C）对线粒体靶向的影响。仅H20P未靶向，并且在创建的8个原卟啉原氧化酶-GFP嵌合融合蛋白中，发现N末端1-17残基是有效线粒体靶向所需的最小原卟啉原氧化酶序列。删除此N端序列显示线粒体定位，表明内部线粒体靶向信号。另外，工程改造了六个构建体以评估电荷和螺旋度对蛋白质线粒体靶向的影响。在那些工程改造者中，只有PPOX20 / H20P-GFP构建体废除了线粒体靶向，大概是通过破坏原卟啉原氧化酶α-螺旋。根据我们的结果，我们提出了一种原卟啉原氧化酶靶向线粒体的机制。

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