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The functional anatomy of basal ganglia disorders
Sp1 and TAFII130 Transcriptional Activity Disrupted in Early Huntington's Disease
TLDR
Coexpression of Sp1 and TAFII130 in cultured striatal cells from wild-type and HD transgenic mice reverses the transcriptional inhibition of the dopamine D2 receptor gene caused by mutant huntingtin, as well as protects neurons from huntingtin-induced cellular toxicity.
Regional and cellular gene expression changes in human Huntington's disease brain.
TLDR
It is concluded that mRNA changes are not attributable to cell loss alone, and data from bona fide HD brains comprise an important reference for hypotheses related to HD and other neurodegenerative diseases.
Metabotropic glutamate receptor mRNA expression in the basal ganglia of the rat
TLDR
MGluR1 was the only mGluR message prominently expressed in the dopaminergic neurons of the substantia nigra pars compacta, suggesting the involvement of this receptor in the regulation of dopamine release from nigrostriatal terminals.
Differential loss of striatal projection neurons in Huntington disease.
TLDR
Analysis of striatal target areas of Huntington disease indicated that in early and middle stages of HD, enkephalin-containing neurons projecting to the external segment of the globus pallidus were much more affected than substance P-containing neuron projections to the internal pallidal segment.
Decreased expression of striatal signaling genes in a mouse model of Huntington's disease.
TLDR
It is demonstrated that mutant huntingtin directly or indirectly reduces the expression of a distinct set of genes involved in signaling pathways known to be critical to striatal neuron function.
Sirtuin 2 inhibitors rescue alpha-synuclein-mediated toxicity in models of Parkinson's disease.
TLDR
A potent inhibitor of sirtuin 2 (SIRT2) was identified and inhibition of SIRT2 rescued alpha-synuclein toxicity and modified inclusion morphology in a cellular model of Parkinson's disease, suggesting a link between neurodegeneration and aging.
A polymorphic DNA marker genetically linked to Huntington's disease
TLDR
The chromosomal localization of the Huntington's disease gene is the first step in using recombinant DNA technology to identify the primary genetic defect in this disorder.
Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease
TLDR
Evidence of caspase-1 activation in the brains of mice and humans with Huntington's disease is demonstrated and it is demonstrated that intracerebroventricular administration of a casp enzyme inhibitor delays disease progression and mortality in the mouse model of Huntington’s disease.
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