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Título : | Frataxin Structure and Function | Autor : | Castro, Ignacio Hugo Pignataro, María Florencia Sewell, Karl Ellioth Espeche, Lucía Daniela Herrera, María Georgina Noguera, Martín Ezequiel Dain, Liliana Nadra, Alejandro Daniel Aran, Martín Smal, Clara Gallo, Mariana Santos, Javier |
Palabras clave : | Tipificación Molecular;Proteínas Mitocondriales | Fecha de publicación : | 2019 | Journal: | Sub-cellular biochemistry | Resumen : | Mammalian 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. |
Descripción : | Fil: Dain, Liliana. ANLIS Dr.C.G.Malbrán. Centro Nacional de Genética Médica Dr. Eduardo Castilla; Argentina. |
URI : | http://sgc.anlis.gob.ar/handle/123456789/1517 | ISSN : | 0306-0225 | DOI: | 10.1007/978-3-030-28151-9_13 |
Aparece en las colecciones: | Publicaciones CeNaGeM |
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