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Archivio digitale delle tesi discusse presso l’Università di Pisa

Tesi etd-11272013-142047


Tipo di tesi
Tesi di laurea magistrale
Autore
PUGLISI, RITA
URN
etd-11272013-142047
Titolo
Understanding the mechanisms of iron-sulfur cluster biogenesis and regulation
Dipartimento
CHIMICA E CHIMICA INDUSTRIALE
Corso di studi
CHIMICA
Relatori
relatore Prof. Di Bari, Lorenzo
relatore Prof.ssa Pastore, Annalisa
Parole chiave
  • CD of proteins
  • Fe-S cluster
  • Frataxin
  • Friedreich ataxia
  • isc operon
Data inizio appello
14/12/2013
Consultabilità
Completa
Riassunto
Iron-sulfur clusters are essential prosthetic groups that are formed under the control of complex machineries highly conserved from bacteria to humans. In bacteria, the genes responsible for cluster biogenesis are mostly grouped in three distinct operons, among which is the Isc operon. This is found in most organisms and contains 7-10 genes. Each of the Isc gene products has a close orthologue in eukaryotes.
Abnormal Fe-S protein biogenesis and mitochondrial iron accumulation in hearth and neurones are for instance part of the typical phenotype of a genetic neurodegenerative disease, Friedreich’s Ataxia. This pathology is caused by the deficiency of a mitochondrial protein, frataxin, extremely conserved throughout species. Although the frataxin function is still unclear, it was proved that this protein is able to bind iron in vitro and in vivo so that it was suggested a role as iron storage or as iron chaperone in the Fe-S cluster biosynthesis. To determine frataxin structural features and differences between species, it is fundamental to completely characterise the system and its modification through evolution.
The final aim of this thesis has been to provide evidence to clarify frataxin’s role in the machinery of Fe-S cluster biosynthesis and to point out some frataxin’s structural properties.
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