Protein Domain : IPR017789

Type:  Family Name:  Frataxin
Description:  Frataxin is a mitochondrial protein, mutation of which leads to the disease Friedreich's ataxia []. Its orthologs are widely distributed in the bacteria, associated with the ISC system for iron-sulphur cluster assembly, and designated CyaY. 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) [, , ]. Short Name:  Frataxin
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0 Child Features

1 Contains

DB identifier Type Name
IPR020895 Conserved_site Frataxin conserved site

1 Cross References

Identifier
TIGR03422

0 Found In

3 GO Annotations

GO Term Gene Name
GO:0004322 IPR017789
GO:0055114 IPR017789
GO:0005739 IPR017789

3 Ontology Annotations

GO Term Gene Name
GO:0004322 IPR017789
GO:0055114 IPR017789
GO:0005739 IPR017789

1 Parent Features

DB identifier Type Name
IPR002908 Family Frataxin/CyaY

384 Proteins

DB identifier UniProt Accession Secondary Identifier Organism Name Length
103203 D8RVZ2 PAC:15407308 Selaginella moellendorffii 109  
29999.m000211 B9SZC1 PAC:16815399 Ricinus communis 200  
Cucsa.119680.1 PAC:16961017 Cucumis sativus 191  
AT4G03240.1 Q9ZR07 PAC:19644849 Arabidopsis thaliana 187  
Thhalv10028999m V4MZG4 PAC:20197150 Eutrema salsugineum 187  
Ciclev10016814m V4UEI0 PAC:20818838 Citrus clementina 196  
Ciclev10016813m V4UEI0 PAC:20818837 Citrus clementina 196  
Ciclev10016799m V4UEI0 PAC:20818836 Citrus clementina 196  
Lus10039728 PAC:23165230 Linum usitatissimum 188  
Lus10033750 PAC:23143270 Linum usitatissimum 183  
Potri.013G150400.1 PAC:26993787 Populus trichocarpa 239  
Gorai.005G207400.1 A0A0D2SKT2 PAC:26806051 Gossypium raimondii 190  
19698 I0YM46 PAC:27388211 Coccomyxa subellipsoidea C-169 110  
9181 C1MW64 PAC:27341230 Micromonas pusilla CCMP1545 115  
107399 C1EBR3 PAC:27401159 Micromonas sp RCC299 190  
30187 A4RSN0 PAC:27414694 Ostreococcus lucimarinus 167  
Thecc1EG007844t1 A0A061E4I3 PAC:27462146 Theobroma cacao 184  
Migut.F00120.1.p A0A022RZC1 PAC:28931626 Mimulus guttatus 199  
Araha.7612s0011.1.p PAC:28842649 Arabidopsis halleri 186  
Cagra.2552s0004.1.p PAC:28895077 Capsella grandiflora 186  
Glyma.03G007300.1.p I1JK59 PAC:30517542 Glycine max 191  
Glyma.19G118800.1.p I1N8F6 PAC:30512794 Glycine max 191  
Brara.C02765.1.p A0A397ZZ50 PAC:30620013 Brassica rapa FPsc 189  
Brara.I00214.1.p A0A397XRV2 PAC:30642717 Brassica rapa FPsc 181  
Bostr.2983s0117.1.p PAC:30662777 Boechera stricta 186  
Cre12.g538350.t1.1 A0A2K3D5B5 PAC:30791905 Chlamydomonas reinhardtii 172  
Medtr6g071520.2 G7KHT5 PAC:31102306 Medicago truncatula 188  
Medtr6g071520.1 G7KHT5 PAC:31102307 Medicago truncatula 188  
Medtr6g071700.1 G7KHU6 PAC:31104600 Medicago truncatula 224  
Medtr7g087030.1 G7L2K8 PAC:31084020 Medicago truncatula 188  

4 Publications

First Author Title Year Journal Volume Pages PubMed ID
            9241270
            8815938
            8596916
            8931268