6qe3

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Re-refinement of 6ESR human IBA57 at 1.75 A resolution

Structural highlights

6qe3 is a 1 chain structure with sequence from Homo sapiens. This structure supersedes the now removed PDB entry 6esr. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:X-ray diffraction, Resolution 1.75Å
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

CAF17_HUMAN Hypotonia-cerebral atrophy-hyperglycinemia syndrome. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry.

Function

CAF17_HUMAN Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway.[1]

Publication Abstract from PubMed

This article describes the approach used to solve the structure of human IBA57 in-house by 5-amino-2,4,6-triiodoisophthalic acid (I3C) high-energy-remote single-wavelength anomalous dispersion (SAD) phasing. Multiple orientations of the same triclinic crystal were exploited to acquire sufficient real data multiplicity for phasing. How the collection of an in-house native data set and its joint use with the I3C derivative through a SIRAS approach decreases the data multiplicity needed by almost 50% is described. Furthermore, it is illustrated that there is a clear data-multiplicity threshold value for success and failure in phasing, and how adding further data does not significantly affect substructure solution and model building. To our knowledge, this is the only structure present in the PDB that has been solved in-house by remote SAD phasing in space group P1 using only one crystal. All of the raw data used, derived from the different orientations, have been uploaded to Zenodo in order to enable software developers to improve methods for data processing and structure solution, and for educational purposes.

In-house high-energy-remote SAD phasing using the magic triangle: how to tackle the P1 low symmetry using multiple orientations of the same crystal of human IBA57 to increase the multiplicity.,Gourdoupis S, Nasta V, Ciofi-Baffoni S, Banci L, Calderone V Acta Crystallogr D Struct Biol. 2019 Mar 1;75(Pt 3):317-324. doi:, 10.1107/S2059798319000214. Epub 2019 Feb 28. PMID:30950402[2]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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Citations
1 reviews cite this structure
Molecular Basis of Rare Diseases Associated to the Maturation of Mitochondrial [4Fe-4S]-Containing Proteins.
Camponeschi et al. (2022)
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References

  1. ↑ Ajit Bolar N, Vanlander AV, Wilbrecht C, Van der Aa N, Smet J, De Paepe B, Vandeweyer G, Kooy F, Eyskens F, De Latter E, Delanghe G, Govaert P, Leroy JG, Loeys B, Lill R, Van Laer L, Van Coster R. Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy. Hum Mol Genet. 2013 Jul 1;22(13):2590-602. doi: 10.1093/hmg/ddt107. Epub 2013 Mar, 5. PMID:23462291 doi:http://dx.doi.org/10.1093/hmg/ddt107
  2. ↑ Gourdoupis S, Nasta V, Ciofi-Baffoni S, Banci L, Calderone V. In-house high-energy-remote SAD phasing using the magic triangle: how to tackle the P1 low symmetry using multiple orientations of the same crystal of human IBA57 to increase the multiplicity. Acta Crystallogr D Struct Biol. 2019 Mar 1;75(Pt 3):317-324. doi:, 10.1107/S2059798319000214. Epub 2019 Feb 28. PMID:30950402 doi:http://dx.doi.org/10.1107/S2059798319000214

Contents


PDB ID 6qe3

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OCA

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