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Frataxin: a protein in search for a function
Author(s) -
Pastore Annalisa,
Puccio Helene
Publication year - 2013
Publication title -
journal of neurochemistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.75
H-Index - 229
eISSN - 1471-4159
pISSN - 0022-3042
DOI - 10.1111/jnc.12220
Subject(s) - frataxin , ataxia , biogenesis , iron–sulfur cluster , iron binding proteins , mitochondrion , function (biology) , biology , neuroscience , oxidative stress , biochemistry , computational biology , microbiology and biotechnology , aconitase , chemistry , gene , enzyme
Reduced levels of the protein frataxin cause the neurodegenerative disease Friedreich's ataxia. Pathology is associated with disruption of iron–sulfur cluster biosynthesis, mitochondrial iron overload, and oxidative stress. Frataxin is a highly conserved iron‐binding protein present in most organisms. Despite the intense interest generated since the determination of its pathology, identification of the cellular function of frataxin has so far remained elusive. In this review, we revisit the most significant milestones that have led us to our current understanding of frataxin and its functions. The picture that emerges is that frataxin is a crucial element of one of the most essential cellular machines specialized in iron‐sulfur cluster biogenesis. Future developments, therefore, can be expected from further advancements in our comprehension of this machine.