PMID- 24668480 OWN - NLM STAT- MEDLINE DCOM- 20140826 LR - 20161125 IS - 0171-2004 (Print) IS - 0171-2004 (Linking) VI - 220 DP - 2014 TI - Huntington's disease. PG - 357-409 LID - 10.1007/978-3-642-45106-5_14 [doi] AB - Changes in the level and activity of brain-derived neurotrophic factor (BDNF) have been described in a number of neurodegenerative disorders since early 1990s. However, only in Huntington disease (HD) gain- and loss-of-function experiments have mechanistically linked these abnormalities with the genetic defect.In this chapter we will describe how huntingtin protein, whose mutation causes HD, is involved in the physiological control of BDNF synthesis and transport in neurons and how both processes are simultaneously disrupted in HD. We will describe the underlying molecular mechanisms and discuss pre-clinical data concerning the impact of the experimental manipulation of BDNF levels on HD progression. These studies have revealed that a major loss of BDNF protein in the brain of HD patients may contribute to the clinical manifestations of the disease. The experimental strategies under investigation to increase brain BDNF levels in animal models of HD will also be described, with a view to ultimately improving the clinical treatment of this condition. FAU - Zuccato, Chiara AU - Zuccato C AD - Department of Biosciences and Centre for Stem cell Research, Universita degli Studi di Milano, Via Viotti 3/5, 20133, Milan, Italy, chiara.zuccato@unimi.it. FAU - Cattaneo, Elena AU - Cattaneo E LA - eng PT - Journal Article PT - Review PL - Germany TA - Handb Exp Pharmacol JT - Handbook of experimental pharmacology JID - 7902231 RN - 0 (Brain-Derived Neurotrophic Factor) RN - 0 (HTT protein, human) RN - 0 (Huntingtin Protein) RN - 0 (Nerve Tissue Proteins) SB - IM MH - Animals MH - Brain Chemistry MH - Brain-Derived Neurotrophic Factor/analysis/*genetics MH - Genetic Therapy MH - Humans MH - Huntingtin Protein MH - Huntington Disease/*genetics/therapy MH - Mutation MH - Nerve Tissue Proteins/genetics MH - Polymorphism, Genetic MH - Promoter Regions, Genetic EDAT- 2014/03/29 06:00 MHDA- 2014/08/27 06:00 CRDT- 2014/03/27 06:00 PHST- 2014/03/27 06:00 [entrez] PHST- 2014/03/29 06:00 [pubmed] PHST- 2014/08/27 06:00 [medline] AID - 10.1007/978-3-642-45106-5_14 [doi] PST - ppublish SO - Handb Exp Pharmacol. 2014;220:357-409. doi: 10.1007/978-3-642-45106-5_14.