Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease
JM Van Raamsdonk, Z Murphy, EJ Slow… - Human molecular …, 2005 - academic.oup.com
Huntington disease (HD) is an adult onset neurodegenerative disorder that predominantly
affects the striatum and cortex despite ubiquitous expression of mutant huntingtin (htt). Here …
affects the striatum and cortex despite ubiquitous expression of mutant huntingtin (htt). Here …
Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease
CE Wang, S Tydlacka, AL Orr, SH Yang… - Human molecular …, 2008 - academic.oup.com
A number of mouse models expressing mutant huntingtin (htt) with an expanded
polyglutamine (polyQ) domain are useful for studying the pathogenesis of Huntington's …
polyglutamine (polyQ) domain are useful for studying the pathogenesis of Huntington's …
Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice
J Bradford, JY Shin, M Roberts, CE Wang… - Journal of Biological …, 2010 - ASBMB
Huntington disease (HD) is caused by an expansion of the polyglutamine (polyQ) repeat (>
37Q) in huntingtin (htt), and age of onset is inversely correlated with the length of the polyQ …
37Q) in huntingtin (htt), and age of onset is inversely correlated with the length of the polyQ …
Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease
ZX Yu, SH Li, J Evans, A Pillarisetti, H Li… - Journal of …, 2003 - Soc Neuroscience
Huntington's disease (HD) mouse models that express N-terminal huntingtin fragments
show rapid disease progression and have been used for developing therapeutics. However …
show rapid disease progression and have been used for developing therapeutics. However …
Phenotype onset in Huntington's disease knock‐in mice is correlated with the incomplete splicing of the mutant huntingtin gene
NR Franich, MA Hickey, C Zhu… - Journal of …, 2019 - Wiley Online Library
Huntington's disease (HD) is a progressive neurodegenerative disorder caused by an
expanded CAG repeat within the huntingtin (HTT) gene. The Q140 and HdhQ150 knock‐in …
expanded CAG repeat within the huntingtin (HTT) gene. The Q140 and HdhQ150 knock‐in …
Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington disease
JM Van Raamsdonk, J Pearson… - Human molecular …, 2005 - academic.oup.com
Huntington disease (HD) is an adult-onset neurodegenerative disease caused by a toxic
gain of function in the huntingtin (htt) protein. The contribution of wild-type htt function to the …
gain of function in the huntingtin (htt) protein. The contribution of wild-type htt function to the …
Progressive phenotype and nuclear accumulation of an amino-terminal cleavage fragment in a transgenic mouse model with inducible expression of full-length mutant …
Y Tanaka, S Igarashi, M Nakamura, J Gafni… - Neurobiology of …, 2006 - Elsevier
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder
characterized behaviorally by chorea, incoordination, and shortened lifespan and …
characterized behaviorally by chorea, incoordination, and shortened lifespan and …
Selective expression of mutant huntingtin during development recapitulates characteristic features of Huntington's disease
AE Molero, EE Arteaga-Bracho… - Proceedings of the …, 2016 - National Acad Sciences
Recent studies have identified impairments in neural induction and in striatal and cortical
neurogenesis in Huntington's disease (HD) knock-in mouse models and associated …
neurogenesis in Huntington's disease (HD) knock-in mouse models and associated …
Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models
RK Graham, EJ Slow, Y Deng, N Bissada, G Lu… - Neurobiology of …, 2006 - Elsevier
Huntington disease (HD) is a devastating neuropsychiatric disease caused by expansion of
a trinucleotide repeat (CAG) in the HD gene. Neuropathological changes include the …
a trinucleotide repeat (CAG) in the HD gene. Neuropathological changes include the …
Huntington and its role in neuronal degeneration
SH Li, XJ Li - The Neuroscientist, 2004 - journals.sagepub.com
Huntington's disease results from a polyglutamine expansion in the N-terminal region of
huntingtin (htt). This abnormality causes protein aggregation and leads to neurotoxicity …
huntingtin (htt). This abnormality causes protein aggregation and leads to neurotoxicity …
相关搜索
- mutant huntingtin huntington's disease
- mutant huntingtin selective degeneration
- nuclear localization selective degeneration
- mutant huntingtin nuclear localization
- huntingtin gene phenotype onset
- mutant huntingtin neurodegeneration in mice
- mutant huntingtin inducible expression
- selective expression huntington's disease
- mutant huntingtin striatal neurons
- huntingtin gene incomplete splicing
- mutant huntingtin cleavage fragment
- mutant huntingtin progressive phenotype
- mutant huntingtin preferential accumulation
- mutant huntingtin pathogenic mechanism
- mutant huntingtin neurological symptoms
- huntington disease phenotypic severity