The DOI matches its Crossref record; 52 of the 52 references it cites were found in a registry or an index.
PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy
Open accessAbstract
The coactivator PGC-1α mediates key responses of skeletal muscle to motor nerve activity. We show here that neuregulin-stimulated phosphorylation of PGC-1α and GA-binding protein (GABP) allows recruitment of PGC-1α to the GABP complex and enhances transcription of a broad neuromuscular junction gene program. Since a subset of genes controlled by PGC-1α and GABP is dysregulated in Duchenne muscular dystrophy (DMD), we examined the effects of transgenic PGC-1α in muscle of mdx mice. These animals show improvement in parameters characteristic of DMD, including muscle histology, running performance, and plasma creatine kinase levels. Thus, control of PGC-1α levels in skeletal muscle could represent a novel avenue to prevent or treat DMD.
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Self-citationTranscriptional co-activator PGC-1α drives the formation of slow-twitch muscle fibres
Lin, J., Wu, H., Tarr, P. T., Zhang, C.-Y., Wu, Z., Boss, O., Michael, L. F., Puigserver, P., Isotani, E., Olson, E. N., et al. (2002) · Nature · doi:10.1038/nature00904 · Cited by 2,546
Self-citationSt-Pierre, J., Drori, S., Uldry, M., Silvaggi, J. M., Rhee, J., Jäger, S., Handschin, C., Zheng, K., Lin, J., Yang, W., et al. (2006) · Cell · doi:10.1016/j.cell.2006.09.024 · Cited by 2,233
Self-citationX chromosome-linked muscular dystrophy (mdx) in the mouse
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Defects in Adaptive Energy Metabolism with CNS-Linked Hyperactivity in PGC-1α Null Mice
Lin, J., Wu, P.-H., Tarr, P. T., Lindenberg, K. S., St-Pierre, J., Zhang, C.-Y., Mootha, V. K., Jäger, S., Vianna, C. R., Reznick, R. M., et al. (2004) · Cell · doi:10.1016/j.cell.2004.09.013 · Cited by 1,162
Self-citationAdvances in Male Contraception
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Sandri, M., Lin, J., Handschin, C., Yang, W., Arany, Z. P., Lecker, S. H., Goldberg, A. L., & Spiegelman, B. M. (2006) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0607795103 · Cited by 974
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Exercise induces transient transcriptional activation of the PGC‐1α gene in human skeletal muscle
Pilegaard, H., Saltin, B., & Neufer, P. D. (2003) · The Journal of Physiology · doi:10.1113/jphysiol.2002.034850 · Cited by 885
Cytokine Stimulation of Energy Expenditure through p38 MAP Kinase Activation of PPARγ Coactivator-1
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“We therefore mutated the known p38 MAPK phosphorylation sites on GABPA (Thr 280) and GABPB1 (Ser 170 and Thr 180) (Fromm and Burden 2001) and used these phosphorylation mutants along with the PGC-1 allele with mutated MAPK phosphorylation sites (PGC1 3A, Thr 262, Ser 265, and Thr 298) (Puigserver et al. 2001) in repor…”
BackgroundMethodInfluential
Cited by
AMP-activated protein kinase (AMPK) action in skeletal muscle via direct phosphorylation of PGC-1α
Jäger, S., Handschin, C., St.-Pierre, J., & Spiegelman, B. M. (2007) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0705070104 · Cited by 2,491
Transcriptional paradigms in mammalian mitochondrial biogenesis and function
Scarpulla, R. C. (2008) · Physiological Reviews · doi:10.1152/physrev.00025.2007 · Cited by 1,666
Cancer Cachexia: Mediators, Signaling, and Metabolic Pathways
Fearon, K. C. H., Glass, D. J., & Guttridge, D. C. (2012) · Cell Metabolism · doi:10.1016/j.cmet.2012.06.011 · Cited by 1,205
The role of exercise and PGC1α in inflammation and chronic disease
Handschin, C., & Spiegelman, B. M. (2008) · Nature · doi:10.1038/nature07206 · Cited by 1,083
Self-citationMitochondrial fragmentation in neurodegeneration.
Knott, A. B., Perkins, G., Schwarzenbacher, R., & Bossy-Wetzel, E. (2008) · Nature Reviews Neuroscience · doi:10.1038/nrn2417 · Cited by 1,002
Metadata
- Authors
- Handschin, C., Kobayashi, Y. M., Chin, S., Seale, P., Campbell, K. P., & Spiegelman, B. M.
- Venue
- Genes & Development
- Publisher
- Cold Spring Harbor Laboratory
- Published
- 2007-04-01
- Volume
- 21
- Issue
- 7
- Pages
- 770-783
- ISSN
- 0890-9369 (ISSN-L)
- 1549-5477
- OpenAlex
- W6968780977
- Cited by
- 357
- References
- 52
- Type
- posted content
- Language
- en
- Open access
- diamond
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- CoChat. (2026). Citation check: PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. https://app.cochat.ai/papers/10.1101/gad.1525107
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- Handschin, C., Kobayashi, Y. M., Chin, S., Seale, P., Campbell, K. P., & Spiegelman, B. M. (2007). PGC-1α regulates the neuromuscular junction program and ameliorates Duchenne muscular dystrophy. Genes & Development. https://doi.org/10.1101/gad.1525107
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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