Article
Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin.
Human molecular genetics - 1 Mar 1999
Schilling G, Becher M W, Sharp A H, Jinnah H A, Duan K, Kotzuk J A, Slunt H H, Ratovitski T, Cooper J K, Jenkins N A, Copeland N G, Price D L, Ross C A, Borchelt D R
Abstract excerpt
Huntington's disease (HD) is an inherited, neurodegenerative disorder caused by the expansion of a glutamine repeat in the N-terminus of the huntingtin protein. To gain insight into the pathogenesis of HD, we generated transgenic mice that express a cDNA encoding an N-terminal fragment (171 amino acids) of huntingtin with 82, 44 or 18 glutamines. Mice expressing relatively low steady-state levels of N171...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.