2m5o

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{{STRUCTURE_2m5o| PDB=2m5o | SCENE= }}
 
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===Solution NMR Structure CTD domain of NFU1 Iron-Sulfur Cluster Scaffold Homolog from Homo sapiens, Northeast Structural Genomics Consortium (NESG) Target HR2876C===
 
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==Disease==
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==Solution NMR Structure CTD domain of NFU1 Iron-Sulfur Cluster Scaffold Homolog from Homo sapiens, Northeast Structural Genomics Consortium (NESG) Target HR2876C==
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[[http://www.uniprot.org/uniprot/NFU1_HUMAN NFU1_HUMAN]] Fatal multiple mitochondrial dysfunction syndrome;Fatal infantile encephalopathy-pulmonary hypertension syndrome. Multiple mitochondrial dysfunctions syndrome 1 (MMDS1) [MIM:[http://omim.org/entry/605711 605711]]: A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:21944046</ref> <ref>PMID:22077971</ref>
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<StructureSection load='2m5o' size='340' side='right'caption='[[2m5o]]' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[2m5o]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2M5O OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2M5O FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/NFU1_HUMAN NFU1_HUMAN]] Iron-sulfur cluster scaffold protein which can assemble [4Fe-2S] clusters and deliver them to target proteins.<ref>PMID:12886008</ref>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2m5o FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2m5o OCA], [https://pdbe.org/2m5o PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2m5o RCSB], [https://www.ebi.ac.uk/pdbsum/2m5o PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2m5o ProSAT]</span></td></tr>
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==About this Structure==
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</table>
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[[2m5o]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2M5O OCA].
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== Disease ==
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[https://www.uniprot.org/uniprot/NFU1_HUMAN NFU1_HUMAN] Fatal multiple mitochondrial dysfunction syndrome;Fatal infantile encephalopathy-pulmonary hypertension syndrome. Multiple mitochondrial dysfunctions syndrome 1 (MMDS1) [MIM:[https://omim.org/entry/605711 605711]: A severe disorder of systemic energy metabolism, resulting in weakness, respiratory failure, lack of neurologic development, lactic acidosis, hyperglycinemia and early death. Some patients show failure to thrive, pulmonary hypertension, hypotonia and irritability. Biochemical features include severe combined deficiency of the 2-oxoacid dehydrogenases, defective lipoic acid synthesis and reduction in activity of mitochondrial respiratory chain complexes. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:21944046</ref> <ref>PMID:22077971</ref>
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==Reference==
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== Function ==
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<references group="xtra"/><references/>
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[https://www.uniprot.org/uniprot/NFU1_HUMAN NFU1_HUMAN] Iron-sulfur cluster scaffold protein which can assemble [4Fe-2S] clusters and deliver them to target proteins.<ref>PMID:12886008</ref>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Acton, T B.]]
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[[Category: Large Structures]]
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[[Category: Everett, J K.]]
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[[Category: Acton TB]]
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[[Category: Hamilton, K.]]
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[[Category: Everett JK]]
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[[Category: Huang, Y J.]]
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[[Category: Hamilton K]]
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[[Category: Janjua, H.]]
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[[Category: Huang YJ]]
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[[Category: Kohan, E.]]
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[[Category: Janjua H]]
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[[Category: Liu, G.]]
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[[Category: Kohan E]]
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[[Category: Montelione, G T.]]
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[[Category: Liu G]]
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[[Category: NESG, Northeast Structural Genomics Consortium.]]
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[[Category: Montelione GT]]
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[[Category: Pederson, K.]]
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[[Category: Pederson K]]
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[[Category: Shastry, R.]]
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[[Category: Shastry R]]
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[[Category: Xiao, R.]]
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[[Category: Xiao R]]
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[[Category: Biosynthetic protein]]
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[[Category: Nesg]]
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[[Category: Protein northeast structural genomics consortium]]
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[[Category: Protein structure initiative]]
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[[Category: Psi-biology]]
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[[Category: Structural genomic]]
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Current revision

Solution NMR Structure CTD domain of NFU1 Iron-Sulfur Cluster Scaffold Homolog from Homo sapiens, Northeast Structural Genomics Consortium (NESG) Target HR2876C

PDB ID 2m5o

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