Glutamine/proline-rich PQE-1 proteins protect Caenorhabditis elegans neurons from huntingtin polyglutamine neurotoxicity

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Huntingtin-interacting protein 1 influences worm and mouse presynaptic function and protects Caenorhabditis elegans neurons against mutant polyglutamine toxicity.

Huntingtin-interacting protein 1 (HIP1) was identified through its interaction with htt (huntingtin), the Huntington's disease (HD) protein. HIP1 is an endocytic protein that influences transport and function of AMPA and NMDA receptors in the brain. However, little is known about its contribution to neuronal dysfunction in HD. We report that the Caenorhabditis elegans HIP1 homolog hipr-1 modula...

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Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity.

Expansion of a polyglutamine tract in the huntingtin protein causes neuronal degeneration and death in Huntington's disease patients, but the molecular mechanisms underlying polyglutamine-mediated cell death remain unclear. Previous studies suggest that expanded polyglutamine tracts alter transcription by sequestering glutamine rich transcriptional regulatory proteins, thereby perturbing their ...

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Salidroside protects Caenorhabditis elegans neurons from polyglutamine-mediated toxicity by reducing oxidative stress.

Polyglutamine (polyQ) aggregation plays a pivotal role in the pathological process of Huntington's disease and other polyQ disorders. Therefore, strategies aiming at restoring dysfunction and reducing stresses mediated by polyQ toxicity are of therapeutic interest for proteotoxicity diseases. Salidroside, a glycoside from Rhodiola rosea, has been shown to have a variety of bioactivities, includ...

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Mutations in the pqe-1 Gene Enhance Transgene Expression in Caenorhabditis elegans

Although various genetic tools have been developed and used as transgenes, the expression of the transgenes often is hampered by negative regulators. Disrupting such negative regulators of gene expression is potentially a way to overcome the common problem of low expression of transgenes. To find such regulators whose mutations enhance transgene expression in Caenorhabditis elegans, we took adv...

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Objective: Oxidative stress that damages proteins result in aging and age related diseases. The aim of this study is to determine the effect of tocotrienol rich fraction (TRF) on the expression of proteins in oxidative stress-induced caenohabditis elegans (C.elegans) which has homologous genes to humans. Methods: The worms were treated with TRF prior to, after and continuously in separate group...

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ژورنال

عنوان ژورنال: Proceedings of the National Academy of Sciences

سال: 2002

ISSN: 0027-8424,1091-6490

DOI: 10.1073/pnas.262544899