Matches in UGent Biblio for { <https://biblio.ugent.be/publication/715852#aggregation> ?p ?o. }
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- aggregation classification "A1".
- aggregation creator B192862.
- aggregation creator B192863.
- aggregation creator B192864.
- aggregation creator person.
- aggregation creator person.
- aggregation creator person.
- aggregation creator person.
- aggregation creator person.
- aggregation date "2009".
- aggregation format "application/pdf".
- aggregation hasFormat 715852.bibtex.
- aggregation hasFormat 715852.csv.
- aggregation hasFormat 715852.dc.
- aggregation hasFormat 715852.didl.
- aggregation hasFormat 715852.doc.
- aggregation hasFormat 715852.json.
- aggregation hasFormat 715852.mets.
- aggregation hasFormat 715852.mods.
- aggregation hasFormat 715852.rdf.
- aggregation hasFormat 715852.ris.
- aggregation hasFormat 715852.txt.
- aggregation hasFormat 715852.xls.
- aggregation hasFormat 715852.yaml.
- aggregation isPartOf urn:issn:0167-4889.
- aggregation language "eng".
- aggregation publisher "Elsevier Science".
- aggregation subject "Medicine and Health Sciences".
- aggregation title "alpha-Skeletal muscle actin nemaline myopathy mutants cause cell death in cultured muscle cells.".
- aggregation abstract "Nemaline myopathy is a neuromuscular disorder, characterized by muscle weakness and hypotonia and is, in 20% of the cases, caused by mutations in the gene encoding alpha-skeletal muscle actin, ACTA1. It is a heterogeneous disease with various clinical phenotypes and severities. In patients the ultrastructure of muscle cells is often disturbed by nemaline rods and it is thought this is the cause for muscle weakness. To search for possible defects during muscle cell differentiation we expressed alpha-actin mutants in myoblasts and allowed these cells to differentiate into myotubes. Surprisingly, we observed two striking new phenotypes in differentiating myoblasts: rounding up of cells and bleb formation, two features reminiscent of apoptosis. Indeed expression of these mutants induced cell death with apoptotic features in muscle cell culture, using AIF and endonuclease G, in a caspase-independent but calpain-dependent pathway. This is the first report on a common cellular defect induced by NM causing actin mutants, independent of their biochemical phenotypes or rod and aggregate formation capacity. These data suggest that lack of type II fibers or atrophy observed in nemaline myopathy patients may be also due to an increased number of dying muscle cells.".
- aggregation authorList BK453602.
- aggregation endPage "1271".
- aggregation issue "7".
- aggregation startPage "1259".
- aggregation volume "1793".
- aggregation aggregates 3112810.
- aggregation isDescribedBy 715852.
- aggregation similarTo j.bbamcr.2009.04.004.
- aggregation similarTo LU-715852.