Unknown,Transcriptomics,Genomics,Proteomics

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Transcription profiling of two different mouse models of Huntington's disease - changes caused by Hdh CAG mutation or 3-nitropropionic acid in striatal cells


ABSTRACT: Affymetrix MG430 2.0 expression levels of wild-type (STHdhQ7/Q7), 3NP-treated wild-type (STHdhQ7/Q7NP), and mutant (STHdhQ111/Q111) striatal cells Experiment Overall Design: Investigation of HD disease mechanism by gene expression profiling of two HD model systems

ORGANISM(S): Mus musculus

DISEASE(S): normal

SUBMITTER: Marcy MacDonald 

PROVIDER: E-GEOD-3583 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications

Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism.

Lee Jong-Min JM   Ivanova Elena V EV   Seong Ihn Sik IS   Cashorali Tanya T   Kohane Isaac I   Gusella James F JF   MacDonald Marcy E ME  

PLoS genetics 20070627 8


The Huntington's disease (HD) CAG repeat, encoding a polymorphic glutamine tract in huntingtin, is inversely correlated with cellular energy level, with alleles over approximately 37 repeats leading to the loss of striatal neurons. This early HD neuronal specificity can be modeled by respiratory chain inhibitor 3-nitropropionic acid (3-NP) and, like 3-NP, mutant huntingtin has been proposed to directly influence the mitochondrion, via interaction or decreased PGC-1alpha expression. We have teste  ...[more]

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