| Article ID | Journal | Published Year | Pages | File Type |
|---|---|---|---|---|
| 6022549 | Neurobiology of Disease | 2012 | 11 Pages |
Abstract
⺠Adequate autonomic control of breathing is chronically missed in CCHS. ⺠PHOX2B polyAla expansions are responsible for intracellular pathogenetic aggregates in CCHS. ⺠No treatment is known to reduce cell aggregates and/or to ameliorate patients' respiration. ⺠The ability of candidate drugs to rescue the correct cellular phenotype has been tested. ⺠17-AAG and curcumin had the most beneficial effects on cell response to expanded PHOX2B.
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Authors
Eleonora Di Zanni, Tiziana Bachetti, Sara Parodi, Paola Bocca, Ignazia Prigione, Simona Di Lascio, Diego Fornasari, Roberto Ravazzolo, Isabella Ceccherini,