Frataxin Structure and Function

dc.creatorCastro, Ignacio Hugo
dc.creatorPignataro, María Florencia
dc.creatorSewell, Karl Ellioth
dc.creatorEspeche, Lucía Daniela
dc.creatorHerrera, María Georgina
dc.creatorNoguera, Martín Ezequiel
dc.creatorDain, Liliana
dc.creatorNadra, Alejandro Daniel
dc.creatorAran, Martín
dc.creatorSmal, Clara
dc.creatorGallo, Mariana
dc.creatorSantos, Javier
dc.date2020-01-22T15:24:56Z
dc.date2020-01-22T15:24:56Z
dc.date2019
dc.date.accessioned2023-08-29T20:06:42Z
dc.date.available2023-08-29T20:06:42Z
dc.identifier0306-0225
dc.identifierhttp://sgc.anlis.gob.ar/handle/123456789/1517
dc.identifier10.1007/978-3-030-28151-9_13
dc.identifier.urihttps://repositorioslatinoamericanos.uchile.cl/handle/2250/8519266
dc.descriptionFil: Dain, Liliana. ANLIS Dr.C.G.Malbrán. Centro Nacional de Genética Médica Dr. Eduardo Castilla; Argentina.
dc.descriptionMammalian frataxin is a small mitochondrial protein involved in iron sulfur cluster assembly. Frataxin deficiency causes the neurodegenerative disease Friedreich's Ataxia. Valuable knowledge has been gained on the structural dynamics of frataxin, metal-ion-protein interactions, as well as on the effect of mutations on protein conformation, stability and internal motions. Additionally, laborious studies concerning the enzymatic reactions involved have allowed for understanding the capability of frataxin to modulate Fe-S cluster assembly function. Remarkably, frataxin biological function depends on its interaction with some proteins to form a supercomplex, among them NFS1 desulfurase and ISCU, the scaffolding protein. By combining multiple experimental tools including high resolution techniques like NMR and X-ray, but also SAXS, crosslinking and mass-spectrometry, it was possible to build a reliable model of the structure of the desulfurase supercomplex NFS1/ACP-ISD11/ISCU/frataxin. In this chapter, we explore these issues showing how the scientific view concerning frataxin structure-function relationships has evolved over the last years.
dc.languageen
dc.relationSub-cellular biochemistry
dc.rightsnone
dc.subjectTipificación Molecular
dc.subjectProteínas Mitocondriales
dc.titleFrataxin Structure and Function
dc.typeOtro tipo de publicación


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