Article
Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin.
Nature medicine - 1 Dec 2009
Okamoto Shu-ichi, Pouladi Mahmoud A, Talantova Maria, Yao Dongdong, Xia Peng, Ehrnhoefer Dagmar E, Zaidi Rameez, Clemente Arjay, Kaul Marcus, Graham Rona K, Zhang Dongxian, Vincent Chen H-S, Tong Gary, Hayden Michael R, Lipton Stuart A
Abstract excerpt
Huntington's disease is caused by an expanded CAG repeat in the gene encoding huntingtin (HTT), resulting in loss of striatal and cortical neurons. Given that the gene product is widely expressed, it remains unclear why neurons are selectively targeted. Here we show the relationship between synaptic and extrasynaptic activity, inclusion formation of mutant huntingtin protein (mtHtt) and neuronal survival....
Topics
- Animals
- Cell Death
- Chromosomes, Artificial, Yeast
- Huntingtin Protein
- Memantine
- Mice
- Mice, Transgenic
- Mutation
- Nerve Tissue Proteins
- Neurons