| pubmed-article:18073330 | rdf:type | pubmed:Citation | lld:pubmed |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C0033684 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C1869123 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C0017262 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C1413113 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C1413357 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C1521761 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C0851285 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C1314939 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C2911684 | lld:lifeskim |
| pubmed-article:18073330 | lifeskim:mentions | umls-concept:C0185117 | lld:lifeskim |
| pubmed-article:18073330 | pubmed:issue | 5 | lld:pubmed |
| pubmed-article:18073330 | pubmed:dateCreated | 2008-5-2 | lld:pubmed |
| pubmed-article:18073330 | pubmed:abstractText | Limb-girdle muscular dystrophy type 2A (LGMD2A) is a recessive genetic disorder caused by mutations in the cysteine protease calpain 3 (CAPN3) that leads to selective muscle wasting. We previously showed that CAPN3 deficiency is associated with a profound perturbation of the NF-kappaB/IkappaB alpha survival pathway. In this study, the consequences of altered NF-kappaB/IkappaB alpha pathway were investigated using biological materials from LGMD2A patients. We first show that the antiapoptotic factor cellular-FLICE inhibitory protein (c-FLIP), which is dependent on the NF-kappaB pathway in normal muscle cells, is down-regulated in LGMD2A biopsies. In muscle cells isolated from LGMD2A patients, NF-kappaB is readily activated on cytokine induction as shown by an increase in its DNA binding activity. However, we observed discrepant transcriptional responses depending on the NF-kappaB target genes. IkappaB alpha is expressed following NF-kappaB activation independent of the CAPN3 status, whereas expression of c-FLIP is obtained only when CAPN3 is present. These data lead us to postulate that CAPN3 intervenes in the regulation of the expression of NF-kappaB-dependent survival genes to prevent apoptosis in skeletal muscle. Deregulations in the NF-kappaB pathway could be part of the mechanism responsible for the muscle wasting resulting from CAPN3 deficiency. | lld:pubmed |
| pubmed-article:18073330 | pubmed:language | eng | lld:pubmed |
| pubmed-article:18073330 | pubmed:journal | http://linkedlifedata.com/r... | lld:pubmed |
| pubmed-article:18073330 | pubmed:citationSubset | IM | lld:pubmed |
| pubmed-article:18073330 | pubmed:chemical | http://linkedlifedata.com/r... | lld:pubmed |
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| pubmed-article:18073330 | pubmed:status | MEDLINE | lld:pubmed |
| pubmed-article:18073330 | pubmed:month | May | lld:pubmed |
| pubmed-article:18073330 | pubmed:issn | 1530-6860 | lld:pubmed |
| pubmed-article:18073330 | pubmed:author | pubmed-author:LochmullerHan... | lld:pubmed |
| pubmed-article:18073330 | pubmed:author | pubmed-author:RichardIsabel... | lld:pubmed |
| pubmed-article:18073330 | pubmed:author | pubmed-author:BaghdiguianSt... | lld:pubmed |
| pubmed-article:18073330 | pubmed:author | pubmed-author:DanieleNathal... | lld:pubmed |
| pubmed-article:18073330 | pubmed:author | pubmed-author:LefrancGérard... | lld:pubmed |
| pubmed-article:18073330 | pubmed:author | pubmed-author:SuelLaurenceL | lld:pubmed |
| pubmed-article:18073330 | pubmed:author | pubmed-author:RaynaudFabric... | lld:pubmed |
| pubmed-article:18073330 | pubmed:author | pubmed-author:BourgNathalie... | lld:pubmed |
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| pubmed-article:18073330 | pubmed:author | pubmed-author:GicquelEvelyn... | lld:pubmed |
| pubmed-article:18073330 | pubmed:issnType | Electronic | lld:pubmed |
| pubmed-article:18073330 | pubmed:volume | 22 | lld:pubmed |
| pubmed-article:18073330 | pubmed:owner | NLM | lld:pubmed |
| pubmed-article:18073330 | pubmed:authorsComplete | Y | lld:pubmed |
| pubmed-article:18073330 | pubmed:pagination | 1521-9 | lld:pubmed |
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| pubmed-article:18073330 | pubmed:year | 2008 | lld:pubmed |
| pubmed-article:18073330 | pubmed:articleTitle | NF-kappaB-dependent expression of the antiapoptotic factor c-FLIP is regulated by calpain 3, the protein involved in limb-girdle muscular dystrophy type 2A. | lld:pubmed |
| pubmed-article:18073330 | pubmed:affiliation | Généthon CNRS FRE3018, 1, 91000 Evry, France. | lld:pubmed |
| pubmed-article:18073330 | pubmed:publicationType | Journal Article | lld:pubmed |
| pubmed-article:18073330 | pubmed:publicationType | Research Support, Non-U.S. Gov't | lld:pubmed |
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