Department of Chemistry, The University of Alabama, Tuscaloosa, AL 35487, USA.
Biochemistry. 2012 Sep 4;51(35):6889-91. doi: 10.1021/bi300779f. Epub 2012 Aug 20.
The progressive neurodegenerative disease Friedreich's ataxia is caused by a decreased level of expression of frataxin, a putative iron chaperone. Frataxin is thought to transiently interact with ISU, the scaffold protein onto which iron-sulfur clusters are assembled, to deliver ferrous iron. Photoreactive heterotrifunctional chemical cross-linking confirmed the interaction between frataxin and ISU in the presence of iron and validated that transient interactions can be covalently trapped with this method. Because frataxin may participate in transient interactions with other mitochondrial proteins, this cross-linking approach may reveal new protein partners and pathways in which it interacts and help deduce direct, downstream consequences of its deficiency.
进行性神经退行性疾病弗里德里希共济失调是由铁载体蛋白 frataxin 的表达水平降低引起的。frataxin 被认为与 ISU （铁硫簇组装的支架蛋白）短暂相互作用，以输送亚铁离子。光反应性杂三功能化学交联在存在铁的情况下证实了 frataxin 与 ISU 之间的相互作用，并验证了这种方法可以共价捕获瞬时相互作用。因为 frataxin 可能参与与其他线粒体蛋白的瞬时相互作用，所以这种交联方法可能揭示它相互作用的新的蛋白质伙伴和途径，并有助于推断其缺乏的直接下游后果。
