Selective degeneration in YAC mouse models of Huntington disease
Huntington disease (HD) is one of at least nine polyglutamine disorders caused by a CAG
expansion in the coding region of a disease-causing gene. These disorders are …
expansion in the coding region of a disease-causing gene. These disorders are …
Cholesterol biosynthesis pathway is disturbed in YAC128 mice and is modulated by huntingtin mutation
Our recent analyses of the cholesterol biosynthetic pathway in Huntington's disease (HD)
cells, in the R6/2 huntingtin-fragment mouse model of HD as well as in human tissues have …
cells, in the R6/2 huntingtin-fragment mouse model of HD as well as in human tissues have …
Testicular degeneration in Huntington disease
JM Van Raamsdonk, Z Murphy, DM Selva… - Neurobiology of …, 2007 - Elsevier
Huntington disease (HD) is an adult onset, neurodegenerative disorder that results from
CAG expansion in the HD gene. Recent work has demonstrated testicular degeneration in …
CAG expansion in the HD gene. Recent work has demonstrated testicular degeneration in …
Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain
JM Van Raamsdonk, M Metzler, E Slow, J Pearson… - Neurobiology of …, 2007 - Elsevier
The YAC128 mouse model of Huntington disease (HD) exhibits motor abnormalities,
cognitive dysfunction and selective neuropathology which are similar to the human disease …
cognitive dysfunction and selective neuropathology which are similar to the human disease …
NMDA receptor function and NMDA receptor-dependent phosphorylation of huntingtin is altered by the endocytic protein HIP1
Huntingtin-interacting protein 1 (HIP1) is an endocytic adaptor protein that plays a role in
clathrin-mediated endocytosis and the ligand-induced internalization of AMPA receptors …
clathrin-mediated endocytosis and the ligand-induced internalization of AMPA receptors …
Characterization of neuron-specific huntingtin aggregates in human huntingtin knock-in mice
H Sawada, H Ishiguro, K Nishii, K Yamada… - Neuroscience …, 2007 - Elsevier
Huntington's disease (HD) is caused by a mutation causing expanded polyglutamine tracts
in the N-terminal fragment of huntingtin. A pathological hallmark of HD is the formation of …
in the N-terminal fragment of huntingtin. A pathological hallmark of HD is the formation of …
Mechanisms of Neurodegeneration in Transgenic Models of Huntington's Disease
JMAC Gil - 2007 - search.proquest.com
Huntington's disease (HD) is a devastating neurodegenerative disorder caused by an
expanded CAG repeat in the IT15 gene, which encodes for huntingtin. The disease is …
expanded CAG repeat in the IT15 gene, which encodes for huntingtin. The disease is …
Proteotoxicity of polyglutamine expansion proteins: Cellular mechanisms and their modulation by molecular chaperones
C Behrends - 2007 - edoc.ub.uni-muenchen.de
Proteins are central to all biological processes. To become functionally active, newly
synthesized protein chains must fold into unique three-dimensional conformations. A group …
synthesized protein chains must fold into unique three-dimensional conformations. A group …
[PDF][PDF] Huntingtin 1-586aa fragment is localized in the nucleus and altered by huntingtin phosphorylation at S421
The experiments in this chapter were conceived and designed by me. I analyzed the data,
prepared the figures and wrote the manuscript. I developed all of the techniques, reagents …
prepared the figures and wrote the manuscript. I developed all of the techniques, reagents …
[PDF][PDF] HMG Advance Access published July 5, 2007
Our recent analyses of the cholesterol biosynthetic pathway in Huntington's disease (HD)
cells, the R6/2 huntingtin-fragment mouse model of HD as well as in human tissues has …
cells, the R6/2 huntingtin-fragment mouse model of HD as well as in human tissues has …