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Cited by 438

The DOI matches its Crossref record; 54 of the 54 references it cites were found in a registry or an index.

Interplay of IKK/NF-κB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy

Open access
Acharyya, Swarnali, Villalta, S. Armando, Bakkar, Nadine, Bupha-Intr, Tepmanas, Janssen, Paul M.L., Carathers, Micheal, Li, Zhi-Wei, Beg, Amer A., Ghosh, Sankar, Sahenk, Zarife, Weinstein, Michael, Gardner, Katherine L., Rafael-Fortney, Jill A., Karin, Michael, Tidball, James G., Baldwin, Albert S., Guttridge, Denis C.·2007·Journal of Clinical Investigation

Abstract

Duchenne muscular dystrophy (DMD) is a lethal X-linked disorder associated with dystrophin deficiency that results in chronic inflammation and severe skeletal muscle degeneration. In DMD mouse models and patients, we find that IkappaB kinase/NF-kappaB (IKK/NF-kappaB) signaling is persistently elevated in immune cells and regenerative muscle fibers. Ablation of 1 allele of the p65 subunit of NF-kappaB was sufficient to improve pathology in mdx mice, a model of DMD. In addition, conditional deletion of IKKbeta in mdx mice elucidated that NF-kappaB functions in activated macrophages to promote inflammation and muscle necrosis and in skeletal muscle fibers to limit regeneration through the inhibition of muscle progenitor cells. Furthermore, specific pharmacological inhibition of IKK resulted in improved pathology and muscle function in mdx mice. Collectively, these results underscore the critical role of NF-kappaB in the progression of muscular dystrophy and suggest the IKK/NF-kappaB signaling pathway as a potential therapeutic target for DMD.

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Metadata

Authors
Acharyya, S., Villalta, S. A., Bakkar, N., Bupha-Intr, T., Janssen, P. M. L., Carathers, M., Li, Z.-W., Beg, A. A., Ghosh, S., Sahenk, Z., Weinstein, M., Gardner, K. L., Rafael-Fortney, J. A., Karin, M., Tidball, J. G., Baldwin, A. S., & Guttridge, D. C.
Venue
Journal of Clinical Investigation
Published
2007-04-02
Volume
117
Issue
4
Pages
889-901
ISSN
0021-9738 (ISSN-L)
OpenAlex
W2098874403
Cited by
438
References
54
FWCI
10.06
Type
journal article
Language
en
Open access
bronze

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CoChat. (2026). Citation check: Interplay of IKK/NF-κB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy. https://app.cochat.ai/papers/10.1172/jci30556
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Acharyya, S., Villalta, S. A., Bakkar, N., Bupha-Intr, T., Janssen, P. M. L., Carathers, M., Li, Z.-W., Beg, A. A., Ghosh, S., Sahenk, Z., Weinstein, M., Gardner, K. L., Rafael-Fortney, J. A., Karin, M., Tidball, J. G., Baldwin, A. S., & Guttridge, D. C. (2007). Interplay of IKK/NF-κB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy. Journal of Clinical Investigation. https://doi.org/10.1172/jci30556

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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).

Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.