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Time-resolved study of mutant ferritins assembly

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ESRF Portal2028-01-01 更新2026-04-23 收录
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https://doi.esrf.fr/10.15151/ESRF-ES-2118292950
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NF is a rare disorder caused by the aberrant production of mutated ferritins, leading to iron accumulation in the brain, particularly in the basal ganglia. Despite being characterized by severe extrapyramidal neurological features (such as focal onset dystonia, chorea, and parkinsonism), there is currently no cure for NF. The molecular mechanisms that trigger neurodegeneration and the vulnerability of cells expressing L-ferritin mutants to ferroptosis remain inadequately understood, posing a significant challenge for developing effective treatments. A time resolved study of the stability of mutated ferritins compared with the WT and the characterization of the iron loading process and of iron induced aggregates would give some insight to support early diagnosis and the development of effective therapies.
提供机构:
Universita di Milano, Dipartimento Biochimica e Fisiologia Generali, Via Celoria 26, 20133 Milano, Italy; Universita di Milano, BioSoftMatter Laboratorio, Dip CBBM, Lita - Via F Lli Cervi 93 Mi, 20090 Segrate, Italy; University of Milan, Medical Biotech. and Translational Medicine, Via F.lli Cervi 93, 20090 Milano, Italy
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2028-01-01
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