El CRAI romandrà tancat del 24 de desembre de 2025 al 6 de gener de 2026. La validació de documents es reprendrà a partir del 7 de gener de 2026.
El CRAI permanecerá cerrado del 24 de diciembre de 2025 al 6 de enero de 2026. La validación de documentos se reanudará a partir del 7 de enero de 2026.
From 2025-12-24 to 2026-01-06, the CRAI remain closed and the documents will be validated from 2026-01-07.
 
Miniatura

Fitxers

534664.pdf (1.95 MB)

Tipus de document

Article

Versió

Versió publicada

Data de publicació

2006

Tots els drets reservats

Si us plau utilitzeu sempre aquest identificador per citar o enllaçar aquest document: https://hdl.handle.net/2445/8314

Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase

Títol de la revista

Autors

Director/Tutor

ISSN de la revista

Títol del volum

Recurs relacionat

http://dx.doi.org/10.1172/JCI27607

Resum

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.

Matèries

Corea de Huntington, Degeneració del sistema nerviós, Quimioteràpia

Matèries (anglès)

Huntington's chorea, Neurodegenerative Diseases, Chemotherapy

Citació

Col·leccions

Articles publicats en revistes (Biomedicina)

Citació

BORRELL PAGÈS, Maria, CANALS I COLL, Josep m., CORDELIÈRES, Fabrice p., PARKER, J. alex, PINEDA MARTÍ, José ramón, GRANGE, Ghislaine, BRYSON, Elzbieta a., GUILLERMIER, Martine, HIRSCH, Etienne, HANTRAYE, Philippe, CHEETHAM, Michael e., NÉRI, Christian, ALBERCH I VIÉ, Jordi, BROUILLET, Emmanuel, SAUDOU, Frédéric, HUMBERT, Sandrine. Cystamine and cysteamine increase brain levels of BDNF in Huntington disease via HSJ1b and transglutaminase. _Journal of Clinical Investigation_. 2006. Vol. 116, núm. 5, pàgs. 1410-1424. [consulta: 2 de gener de 2026]. ISSN: 0021-9738. [Disponible a: https://hdl.handle.net/2445/8314]

Exportar metadades

JSON - METS

Compartir registre