Rasheed Masooma, Yan Robert, Kelly Geoff, Pastore Annalisa
Maurice Wohl Institute, King's College London, 5 Cutcombe Rd, London, SE5 9RT, UK.
MRC-NMR Centre, The Crick Institute, London, NW7 1AT, UK.
Biomol NMR Assign. 2018 Apr;12(1):113-116. doi: 10.1007/s12104-017-9790-3. Epub 2017 Oct 31.
Frataxin is the protein responsible for the genetically-inherited neurodegenerative disease Friedreich's ataxia caused by partial silencing of the protein and loss of function. Although the frataxin function is not yet entirely clear, it has been associated to the machine that builds iron-sulfur clusters, essential prosthetic groups involved in several processes and is strongly conserved in organisms from bacteria to humans. Two of its important molecular partners are the protein NFS1 (or IscS in bacteria), that is the desulfurase which converts cysteine to alanine and produces sulfur, and ISU (or IscU), the scaffold protein which transiently accepts the cluster. While bacterial frataxin has been extensively characterized, only few eukaryotic frataxins have been described. Here we report the H, C and N backbone and side-chain chemical shift assignments of frataxin from Chaetomium thermophilum, a thermophile increasingly used by virtue of its stability.
弗里德赖希共济失调蛋白是一种由该蛋白部分沉默和功能丧失引起的遗传性神经退行性疾病——弗里德赖希共济失调的致病蛋白。虽然弗里德赖希共济失调蛋白的功能尚未完全明确，但它与构建铁硫簇的机制有关，铁硫簇是参与多种过程的重要辅基，并且在从细菌到人类的生物体中高度保守。它的两个重要分子伴侣是蛋白质NFS1（在细菌中为IscS），即把半胱氨酸转化为丙氨酸并产生硫的脱硫酶，以及ISU（或IscU），即短暂接受该簇的支架蛋白。虽然细菌弗里德赖希共济失调蛋白已得到广泛表征，但只有少数真核弗里德赖希共济失调蛋白被描述过。在此，我们报告嗜热毛壳菌中弗里德赖希共济失调蛋白的氢、碳和氮主链以及侧链化学位移归属，嗜热毛壳菌是一种因其稳定性而越来越常用的嗜热菌。
