The phylogenetic distribution of frataxin indicates a role in iron-sulfur cluster protein assembly

Human Molecular Genetics

Volumn 10, Issue 21, 2001, Pages 2463-2468

  Huynen, Martijn A ^c   Snel, Berend ^b   Bork, Peer ^a   Gibson, Toby J ^b  

^a MAX DELBRÜCK CENTER FOR MOLECULAR MEDICINE   (Germany)

^b EUROPEAN MOLECULAR BIOLOGY LABORATORY   (Germany)

^c RADBOUD UNIVERSITY MEDICAL CENTER   (Netherlands)

Author keywords

[No Author keywords available]

Indexed keywords

CHAPERONE; FERREDOXIN; FRATAXIN; HEAT SHOCK PROTEIN; HEAT SHOCK PROTEIN 20; HEAT SHOCK PROTEIN 66; HSCA PROTEIN; HSCB PROTEIN; IRON; JAC1 PROTEIN; SSQ1 PROTEIN; SULFUR; UNCLASSIFIED DRUG;

ARTICLE; AUTOSOMAL RECESSIVE DISORDER; BIOCHEMISTRY; CELLULAR DISTRIBUTION; CONTROLLED STUDY; CYTOPATHOLOGY; DEGENERATIVE DISEASE; FRIEDREICH ATAXIA; GENE TRANSFER; GRAM NEGATIVE BACTERIUM; HYPOTHESIS; MITOCHONDRION; MOLECULAR EVOLUTION; NONHUMAN; PHYLOGENY; PREDICTION; PRIORITY JOURNAL; PROTEIN ASSEMBLY; PROTEIN EXPRESSION; PROTEIN PROTEIN INTERACTION; SACCHAROMYCES CEREVISIAE;

ATAXIA; NEGIBACTERIA; PROTEOBACTERIA; SACCHAROMYCES CEREVISIAE;

EID: 0035504444     PISSN: 09646906     EISSN: None     Source Type: Journal    
DOI: 10.1093/hmg/10.21.2463     Document Type: Article

References (47)

1. Gene and context: Integrative approaches to genome analysis
2. Measuring genome evolution
3. Assigning protein functions by comparative genome analysis: Protein phylogenetic profiles
4. Detecting protein function and protein-protein interactions from genome sequences
5. Predicting protein function by genomic context: Quantitative evaluation and qualitative inferences
6. Lineage-specific loss and divergence of functionally linked genes in eukaryotes
7. Modularity in the gain and loss of genes: Applications for function prediction
8. Towards a structural understanding of Friedreich's ataxia: The solution structure of frataxin
9. Crystal structure of Escherichia coli CyaY protein reveals a previously unidentified fold for the evolutionarily conserved frataxin family
10. Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes
11. Clinical and genetic abnormalities in patients with Friedreich's ataxia
12. Recent advances in the molecular pathogenesis of Friedreich's ataxia
13. Low iron concentrations and aconitase deficiency in a yeast frataxin homologue deficient strain
14. Iron-dependent self-assembly of the recombinant yeast frataxin: Implications for Friedreich's ataxia
15. Friedreich's ataxia protein: Phylogenetic evidence for mitochondrial dysfunction
16. Regulation of mitochondrial accumulation by Yfh1p, a putative homolog of frataxin
17. Deletion of the yeast homologue of the human gene associated with Friedreich's ataxia elicits iron accumulation in mitochondria
18. Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue
19. Assembly of iron-sulfur clusters. Identification of an iscSUA-hscBA-fdx gene cluster from Azotobacter vinelandii
20. Functional assignment of the ORF2-iscS-iscU-iscA-hscB-hscA-fdx-ORF3 gene cluster involved in the assembly of Fe-S clusters in Escherichia coli
21. The cysteine desulfurase, IscS, has a major role in in vivo Fe-S cluster formation in Escherichia coli
22. Evidence for a conserved system for iron metabolism in the mitochondria of Saccharomyces cerevisiae
23. Isa1p is a component of the mitochondrial machinery for maturation of cellular iron-sulfur proteins and requires conserved cysteine residues for function
24. Role of Saccharomyces cerevisiae ISA1 and ISA2 in iron homeostatis
25. A mitochondrial ferredoxin is essential for the biogenesis of cellular iron-sulfur proteins
26. The mitochondrial proteins Ssq1 and Jac1 are required for the assembly of iron sulfur clusters in mitochondria
27. Jac1, a mitochondrial J-type chaperone, is involved in the biogenesis of Fe/S clusters in Saccharomyces cerevisiae
28. The mitochondrial proteins Atm1p and Nfs1p are essential for biogenesis of cytosolic Fe/S proteins
29. ARH1 of Saccharomyces cerevisiae: A new essential gene that codes for a protein homologous to the human adrenodoxin reductase
30. Maturation of cellular Fe-S proteins: An essential function of mitochondria
31. Distinct iron-sulfur cluster assembly proteins exist in the cytosol and mitochondria of human cells
32. PSORT: A program for detecting sorting signals in proteins and predicting their subcellular localization
33. Its substrate specificity characterizes the DnaJ co-chaperone as a scanning factor for the DnaK chaperone
34. Crystal structure of Hsc20, a J-type co-chaperone from Escherichia coli
35. The Fe/S assembly protein IscU behaves as a substrate for the molecular chaperone Hsc66 from Escherichia coli
36. IscU as a scaffold for iron-sulfur cluster biosynthesis: Sequential assembly if [2Fe-2S] and [4Fe-4S] clusters in IscU
37. Iron sulfur cluster assembly: Characterization of IscA and evidence for a functional complex with ferredoxin
38. Interaction of the iron-sulfur cluster assembly protein IscU with the Hsc66/Hsc20 molecular chaperone system of Escherichia coli
39. Structure-function analysis of the zinc-finger region of the DnaJ molecular chaperone
40. A zinc finger-like domain of the molecular chaperone DnaJ is involved in binding to denatured protein substrates
41. Role of the mitochondrial Hsp70s, Ssc1 and Ssq1, in the maturation of Yfh1
42. Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits
43. Identification of common molecular subsequences
44. Gapped BLAST and PSI-BLAST: A new generation of protein database search programs
45. Multiple sequence alignment with ClustalX
46. The neighbor-joining method: A new method for reconstructing phylogenetic trees
47. Genome phylogeny based on gene content