Early and brain region-specific decrease of de novo cholesterol biosynthesis in Huntington's disease: A cross-validation study in Q175 knock-in mice

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• 作者：Mahalakshmi Shankaran^a ; Eleonora Di Paolo^b ; Valerio Leoni^c ; ^d ; Claudio Caccia^c ; Costanza Ferrari Bardile^b ; Hussein Mohammed^a ; Stefano Di Donato^c ; ¹ ; Seung Kwak^e ; Deanna Marchionini^e ; Scott Turner^a ; Elena Cattaneo^b ; ^{elena.cattaneo@unimi.it} ; Marta Valenza^b ; ^{marta.valenza@unimi.it}
• 关键词：Huntington ; Brain cholesterol ; Lathosterol ; Striatum
• 刊名：Neurobiology of Disease
• 出版年：2017
• 出版时间：February 2017
• 年：2017
• 卷：98
• 期：Complete
• 页码：66-76
• 全文大小：1573 K
• 卷排序：98

文摘

A new and multi-platform in vivo study show early reduction in cholesterol biosynthesis in the HD mouse striatum. There is a direct correlation between cholesterol synthesis and new 24OHC, the brain-specific cholesterol catabolite. The decrease of cholesterol biosynthesis in the striatum is CAG-length dependent.