| pubmed-article:17092942 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C0026882 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C0009968 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C0019602 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C0205216 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C0669516 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C0023688 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C1167622 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C0205360 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C1709915 | lld:lifeskim |
| pubmed-article:17092942 | lifeskim:mentions | umls-concept:C0596448 | lld:lifeskim |
| pubmed-article:17092942 | pubmed:issue | 1 | lld:pubmed |
| pubmed-article:17092942 | pubmed:dateCreated | 2007-1-1 | lld:pubmed |
| pubmed-article:17092942 | pubmed:databankReference | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:17092942 | pubmed:abstractText | A subset of superoxide dismutase 1 (Cu/Zn-SOD1) mutants that cause familial amyotrophic lateral sclerosis (FALS) have heightened reactivity with (-)ONOO and H(2)O(2) in vitro. This reactivity requires a copper ion bound in the active site and is a suggested mechanism of motor neuron injury. However, we have found that transgenic mice that express SOD1-H46R/H48Q, which combines natural FALS mutations at ligands for copper and which is inactive, develop motor neuron disease. Using a direct radioactive copper incorporation assay in transfected cells and the established tools of single crystal x-ray diffraction, we now demonstrate that this variant does not stably bind copper. We find that single mutations at copper ligands, including H46R, H48Q, and a quadruple mutant H46R/H48Q/H63G/H120G, also diminish the binding of radioactive copper. Further, using native polyacrylamide gel electrophoresis and a yeast two-hybrid assay, the binding of copper was found to be related to the formation of the stable dimeric enzyme. Collectively, our data demonstrate a relationship between copper and assembly of SOD1 into stable dimers and also define disease-causing SOD1 mutants that are unlikely to robustly produce toxic radicals via copper-mediated chemistry. | lld:pubmed |
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| pubmed-article:17092942 | pubmed:language | eng | lld:pubmed |
| pubmed-article:17092942 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:17092942 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:17092942 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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| pubmed-article:17092942 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:17092942 | pubmed:month | Jan | lld:pubmed |
| pubmed-article:17092942 | pubmed:issn | 0021-9258 | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:BorcheltDavid... | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:SluntHilda... | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:HartP JohnPJ | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:WangJiouJ | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:CaoXiaohangX | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:RodriguezAnge... | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:GitlinJonatha... | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:Caruano-Yzerm... | lld:pubmed |
| pubmed-article:17092942 | pubmed:author | pubmed-author:ScheurmannJon... | lld:pubmed |
| pubmed-article:17092942 | pubmed:issnType | Print | lld:pubmed |
| pubmed-article:17092942 | pubmed:day | 5 | lld:pubmed |
| pubmed-article:17092942 | pubmed:volume | 282 | lld:pubmed |
| pubmed-article:17092942 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:17092942 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:17092942 | pubmed:pagination | 345-52 | lld:pubmed |
| pubmed-article:17092942 | pubmed:dateRevised | 2011-1-11 | lld:pubmed |
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| pubmed-article:17092942 | pubmed:meshHeading | pubmed-meshheading:17092942... | lld:pubmed |
