The DOI matches its Crossref record; 1 of the 1 references it cites were found in a registry or an index.
Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase
Open accessAbstract
There is no treatment for the neurodegenerative disorder Huntington disease (HD). Cystamine is a candidate drug; however, the mechanisms by which it operates remain unclear. We show here that cystamine increases levels of the heat shock DnaJ-containing protein 1b (HSJ1b) that are low in HD patients. HSJ1b inhibits polyQ-huntingtin-induced death of striatal neurons and neuronal dysfunction in Caenorhabditis elegans. This neuroprotective effect involves stimulation of the secretory pathway through formation of clathrin-coated vesicles containing brain-derived neurotrophic factor (BDNF). Cystamine increases BDNF secretion from the Golgi region that is blocked by reducing HSJ1b levels or by overexpressing transglutaminase. We demonstrate that cysteamine, the FDA-approved reduced form of cystamine, is neuroprotective in HD mice by increasing BDNF levels in brain. Finally, cysteamine increases serum levels of BDNF in mouse and primate models of HD. Therefore, cysteamine is a potential treatment for HD, and serum BDNF levels can be used as a biomarker for drug efficacy.
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Cites
Mice lacking brain-derived neurotrophic factor develop with sensory deficits
Ernfors, P., Lee, K.-F., & Jaenisch, R. (1994) · Nature · doi:10.1038/368147a0 · Cited by 1,067
Cited by
Brain-derived neurotrophic factor in neurodegenerative diseases
Zuccato, C., & Cattaneo, E. (2009) · Nature Reviews Neurology · doi:10.1038/nrneurol.2009.54 · Cited by 947
Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease
Zuccato, C., Valenza, M., & Cattaneo, E. (2010) · Physiological Reviews · doi:10.1152/physrev.00041.2009 · Cited by 848
The corticostriatal pathway in Huntington's disease
CEPEDA, C., WU, N., ANDRE, V, CUMMINGS, D., & LEVINE, M. (2007) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2006.11.001 · Cited by 310
Impaired TrkB receptor signaling underlies corticostriatal dysfunction in Huntington’s disease
Plotkin, J. L., Day, M., Peterson, J. D., Xie, Z., Kress, G. J., Rafalovich, I., Kondapalli, J., Gertler, T. S., Flajolet, M., Greengard, P., et al. (2014) · Neuron · doi:10.1016/j.neuron.2014.05.032 · Cited by 187
Chiang, M.-C., Chen, H.-M., Lee, Y.-H., Chang, H.-H., Wu, Y.-C., Soong, B.-W., Chen, C.-M., Wu, Y.-R., Liu, C.-S., Niu, D.-M., et al. (2007) · Human Molecular Genetics · doi:10.1093/hmg/ddl481 · Cited by 95
Metadata
- Authors
- Borrell-Pages, M.
- Venue
- Journal of Clinical Investigation
- Published
- 2006-05-01
- Volume
- 116
- Issue
- 5
- Pages
- 1410-1424
- ISSN
- 0021-9738 (ISSN-L)
- OpenAlex
- W2107007950
- Cited by
- 236
- References
- 1
- FWCI
- 5.44
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Borrell-Pages, M. (2006). Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. Journal of Clinical Investigation. https://doi.org/10.1172/jci27607
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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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