{"product_id":"energy-dysregulation-in-huntingtons-disease-von-tz-chuen-ju","title":"Energy Dysregulation in Huntington's Disease","description":"\u003cp\u003eAdenosine monophosphate-activated protein kinase (AMPK) is a major energy sensor that maintains cellular energy homeostasis. Huntington¿s disease (HD) is a neurodegenerative disorder caused by the expansion of CAG repeats in the huntingtin (Htt) gene. Herein, we report that activation of the ¿1 isoform of AMPK (AMPK-¿1) occurred in striatal neurons of humans and mice with HD. Overactivation of AMPK in the striatum caused brain atrophy, facilitated neuronal loss, and increased formation of Htt aggregates in a transgenic mouse model (R6\/2) of HD. Moreover, mHtt induced oxidative stress which caused activation of the a isoform of AMPK, and subsequent neurodegeneration. Oxidative stress played a critical role in the activation of AMPK revealed by the fact that the level of oxidative stress was higher in striatal cells expressing mutant Htt, and that a blocker of oxidative stress suppressed the activation of AMPK. Collectively, activation of AMPK-¿1 played a pivotal role in the potentiation of neurodegeneration.\u003c\/p\u003e\u003cdiv class=\"aw-variant-hidden-subtitle-div\" id=\"aw-variant-subtitle-9783330823372\"\u003e\u003ch3\u003e\u003c\/h3\u003e\u003c\/div\u003e","brand":"Libri","offers":[{"title":"Softcover - 9783330823372","offer_id":39427190095965,"sku":"9783330823372","price":24.8,"currency_code":"EUR","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0940\/0622\/files\/26fa497c-0d81-4d4f-b52b-a5b5ddc1fe2b.jpg?v=1775885937","url":"https:\/\/shop.autorenwelt.de\/products\/energy-dysregulation-in-huntingtons-disease-von-tz-chuen-ju","provider":"Autorenwelt Shop","version":"1.0","type":"link"}