The chemical form of mitochondrial iron in Friedreich's ataxia.
The chemical form of mitochondrial iron in Friedreich's ataxia.
复制标题
弗里德赖希共济失调中线粒体铁的化学形式。
DOI：
10.1016/j.jinorgbio.2007.03.004
复制
发表时间：
2007
影响因子：
3.9
通讯作者：
Nichol,Helen
中科院分区：
文献类型：
--
作者：
Popescu,BogdanFGh;Pickering,IngridJ;George,GrahamN;Nichol,Helen
弗里德赖希共济失调（FRDA）是由线粒体基质蛋白共济失调蛋白缺乏引起的细胞损伤引起的。为了解决共济失调蛋白缺乏对线粒体铁化学的影响，从来自FRDA受影响和未受影响个体的原代成纤维细胞分离重线粒体级分（HMF）。X射线吸收光谱法用于表征铁的化学形式。近K边光谱与一系列模型铁化合物拟合，以确定每种铁物种的比例。受影响和未受影响的成纤维细胞中的大多数铁是水合铁。来自未受影响的HMF的铁K-边缘与由frataxin建模的组织差的水铁矿最佳拟合，而来自受影响的细胞的HMF与由铁蛋白建模的高度组织化的水铁矿最佳拟合。两者都有几个次要的铁物种，但这些并没有不同的疾病一致。由于铁蛋白和frataxin的铁K-边光谱是非常相似的，我们提出了额外的证据，铁蛋白结合铁的HMF的存在。受影响细胞的HMF中的主要铁蛋白亚基在大小和抗原性上类似于线粒体铁蛋白（MtFt）。天然凝胶的蛋白质印迹显示，来自受影响细胞的HMF具有3倍以上的含铁蛋白的全铁蛋白。我们得出结论，受影响和未受影响的细胞的成纤维细胞HMF中的大多数铁是水铁矿，但只有FRDA受影响的细胞在线粒体铁蛋白中矿化显著的铁。
Friedreich’s ataxia (FRDA) results from cellular damage caused by a deficiency in the mitochondrial matrix protein frataxin. To address the effect of frataxin deficiency on mitochondrial iron chemistry, the heavy mitochondrial fraction (HMF) was isolated from primary fibroblasts from FRDA affected and unaffected individuals. X-ray absorption spectroscopy was used to characterize the chemical form of iron. Near K-edge spectra were fitted with a series of model iron compounds to determine the proportion of each iron species. Most of the iron in both affected and unaffected fibroblasts was ferrihydrite. The iron K-edge from unaffected HMFs were best fitted with poorly organized ferrihydrite modeled by frataxin whereas HMFs from affected cells were best fitted with highly organized ferrihydrite modeled by ferritin. Both had several minor iron species but these did not differ consistently with disease. Since the iron K-edge spectra of ferritin and frataxin are very similar, we present additional evidence for the presence of ferritin-bound iron in HMF. The predominant ferritin subunit in HMFs from affected cells resembled mitochondrial ferritin (MtFt) in size and antigenicity. Western blotting of native gels showed that HMF from affected cells had 3-fold more holoferritin containing stainable iron. We conclude that most of the iron in fibroblast HMF from both affected and unaffected cells is ferrihydrite but only FRDA affected cells mineralize significant iron in mitochondrial ferritin.
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影响因子：
3.5
作者：
Anderson, PR;Kirby, K;Phillips, JP
通讯作者：
Phillips, JP
影响因子：
5.6
作者：
Bou-Abdallah, F;Santambrogio, P;Chasteen, ND
通讯作者：
Chasteen, ND
弗里德赖希共济失调的心脏病理学：文献复习及一例报道
DOI：
--
发表时间：
1976
期刊：
Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques
影响因子：
--
作者：
G. Sanchez‐Casis;M. Côté;A. Barbeau
通讯作者：
A. Barbeau
弗里德赖希共济失调，人淋巴母细胞和成纤维细胞中线粒体不稳定铁没有变化
影响因子：
4.8
作者：
B. Sturm;Ute Bistrich;M. Schranzhofer;J. Sarsero;U. Rauen;B. Scheiber;H. de Groot;P. Ioannou;F. Petrat
通讯作者：
F. Petrat
胰岛中的 Frataxin 缺乏会因 β 细胞质量的损失而导致糖尿病。
影响因子：
15.9
作者：
M. Ristow;H. Mulder;D. Pomplun;T. Schulz;Katrin Müller;Anja Krause;M. Fex;H. Puccio;Jörg Müller;F. Isken;J. Spranger;D. Müller;M. Magnuson;M. Möhlig;M. Koenig;A. Pfeiffer
通讯作者：
A. Pfeiffer