Frataxin_Cyay

Frataxin-like domain
Frataxin_Cyay
SMART accession number:SM01219
Description: This family contains proteins that have a domain related to the globular C-terminus of Frataxin the protein that is mutated in Friedreich's ataxia. This domain is found in a family of bacterial proteins. The function of this domain is currently unknown. It has been suggested that this family is involved in iron transport.
Interpro abstract (IPR002908):

The eukaryotic proteins in this entry include frataxin, the protein that is mutated in Friedreich's ataxia [(PUBMED:8931268)], and related sequences. Friedreich's ataxia is a progressive neurodegenerative disorder caused by loss of function mutations in the gene encoding frataxin (FRDA). Frataxin mRNA is predominantly expressed in tissues with a high metabolic rate (including liver, kidney, brown fat and heart). Mouse and yeast frataxin homologues contain a potential N-terminal mitochondrial targeting sequence, and human frataxin has been observed to co-localise with a mitochondrial protein. Furthermore, disruption of the yeast gene has been shown to result in mitochondrial dysfunction. Friedreich's ataxia is thus believed to be a mitochondrial disease caused by a mutation in the nuclear genome (specifically, expansion of an intronic GAA triplet repeat) [(PUBMED:8596916), (PUBMED:8815938), (PUBMED:9241270)].

The bacterial proteins in this entry are iron-sulphur cluster (FeS) metabolism CyaY proteins homologous to eukaryotic frataxin. Partial Phylogenetic Profiling [(PUBMED:16930487)] suggests that CyaY most likely functions as part of the ISC system for FeS cluster biosynthesis, and is supported by expermimental data in some species [(PUBMED:16603772), (PUBMED:16428423)].

GO process:iron-sulfur cluster assembly (GO:0016226)
GO function:ferric iron binding (GO:0008199)
Family alignment:
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There are 4591 Frataxin_Cyay domains in 4591 proteins in SMART's nrdb database.

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