Huntington’s Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy

摘要: Huntington’s disease (HD) is an inherited disorder characterized by neuronal dysfunction and degeneration in striatum cerebral cortex. Although the signaling pathways involved HD are not yet clearly elucidated, mutant huntingtin protein a key factor induction of neurodegeneration. The alters intracellular Ca2+ homeostasis, disrupts trafficking impairs gene transcription. In this review, I emphasize effects handling transcriptional factors. Transcriptional alterations factors deficits several proteins cellular machinery. These include neurotrophic such as brain-derived factor, fibroblast growth glial-cell-line-derived ciliary neurturin that have been suggested to restore dysfunction, improve behavioral prolong survival animal models HD. An understanding molecular neurodegeneration will shed light on choice targeting specific population consequently overcome deficits.