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Resources » Paper

Philipp Christoph Janiesch et al. (2008) European Worm Meeting "The Ubiquitin-Selective Chaperone CDC-48 Links Myosin Assembly in C. elegans to Human Myopathy"

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  • Comments on Philipp Christoph Janiesch et al. (2008) European Worm Meeting "The Ubiquitin-Selective Chaperone CDC-48 Links Myosin Assembly in C. elegans to Human Myopathy" (0)

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    Status:
    Publication type:
    Meeting_abstract
    WormBase ID:
    WBPaper00033291

    Philipp Christoph Janiesch, Johnny Kim, Julien Mouysset, Roja Barikbin, Hanns Lochmuller, Giuseppe Cassata, Sabine Krause, & Thorsten Hoppe, (2008). The Ubiquitin-Selective Chaperone CDC-48 Links Myosin Assembly in C. elegans to Human Myopathy presented in European Worm Meeting. Unpublished information; cite only with author permission.

    Protein degradation in eukaryotes often requires the ubiquitin-selective. chaperone p97 for substrate recruitment and ubiquitin chain assembly.. However, the physiological relevance of p97 and its role in developmental. processes are still unclear. Here, we discovered an unanticipated function. of CDC-48/p97 in myosin assembly and myofibril organization both in C.. elegans and humans. The developmentally regulated assembly of a CDC-48/UFD-. 2/CHN-1 complex links turnover of the myosin-directed chaperone UNC-45 to. functional muscle formation. Our data suggest a similarly conserved pathway. regulating myosin assembly in man. Remarkably, mutations in human p97,. known to cause hereditary inclusion body myopathy, abrogate UNC-45. degradation and result in severely disorganized myofibrils, detrimental. towards sarcomeric function. These results identify a key role of CDC-. 48/p97 in the process of myofiber differentiation and maintenance, which is. abolished during pathological conditions leading to protein aggregation and. inclusion body formation in human skeletal muscle. To our knowledge this is. the first study implicating functional myosin assembly and maintenance in. the pathophysiology of human inclusion body myopathy associated with Paget. disease of bone and frontotemporal dementia (IBMPFD).. The ubiquitin-selective chaperone CDC-48/p97 links myosin assembly to human. myopathy. Janiesch P.C., Kim J., Mouysset J., Barikbin R., Lochmuller H.,. Cassata G., Krause S., and Hoppe T. (2007). Nat. Cell Biol. 9, 379-390.. Multiubiquitylation by E4 enzymes: one size doesn''t fit all.. Hoppe T. (2005). Trends Biochem. Sci. 30, 183-187.

    Authors: Philipp Christoph Janiesch, Johnny Kim, Julien Mouysset, Roja Barikbin, Hanns Lochmuller, Giuseppe Cassata, Sabine Krause, Thorsten Hoppe,


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