Search results for "Huntingtin-associated protein 1"

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Expression of genes encoding the calcium signalosome in cellular and transgenic models of Huntington's disease

2013

Huntington's disease (HD) is a hereditary neurodegenerative disease caused by the expansion of a polyglutamine stretch in the huntingtin (HTT) protein and characterized by dysregulated calcium homeostasis. We investigated whether these disturbances are correlated with changes in the mRNA level of the genes that encode proteins involved in calcium homeostasis and signaling (i.e., the calciosome). Using custom-made TaqMan low-density arrays containing probes for 96 genes, we quantified mRNA in the striatum in YAC128 mice, a model of HD, and wildtype mice. HTT mutation caused the increased expression of some components of the calcium signalosome, including calretinin, presenilin 2, and calmyri…

Huntingtinhuntingtincalcium signalosomechemistry.chemical_elementtransgenic miceCalciumlcsh:RC321-571Cellular and Molecular Neurosciencehuntingtin-associated protein 1mental disordersGene expressionOriginal Research Articlelcsh:Neurosciences. Biological psychiatry. NeuropsychiatryMolecular BiologyCalcium metabolismTaqMan low-density arraysbiologyHuntingtin-associated protein 1Calcium channelTaqMan Low Density Arraysstore-operated calcium entrycalcyclin-binding proteinHuntington's diseaseMolecular biologyStore-operated calcium entrynervous systemchemistrybiology.proteinCalretininHuntington’s diseaseNeuroscienceFrontiers in Molecular Neuroscience
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Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules.

2004

AbstractPolyglutamine expansion (polyQ) in the protein huntingtin is pathogenic and responsible for the neuronal toxicity associated with Huntington's disease (HD). Although wild-type huntingtin possesses antiapoptotic properties, the relationship between the neuroprotective functions of huntingtin and pathogenesis of HD remains unclear. Here, we show that huntingtin specifically enhances vesicular transport of brain-derived neurotrophic factor (BDNF) along microtubules. Huntingtin-mediated transport involves huntingtin-associated protein-1 (HAP1) and the p150Glued subunit of dynactin, an essential component of molecular motors. BDNF transport is attenuated both in the disease context and b…

congenital hereditary and neonatal diseases and abnormalitiesHuntingtinCell SurvivalContext (language use)Nerve Tissue ProteinsMicrotubulesModels BiologicalGeneral Biochemistry Genetics and Molecular BiologyMiceNeurotrophic factorsmental disordersHuntingtin ProteinAnimalsCells CulturedNeuronsHuntingtin ProteinbiologyBiochemistry Genetics and Molecular Biology(all)Huntingtin-associated protein 1Brain-Derived Neurotrophic FactorCytoplasmic VesiclesBrainNuclear ProteinsBiological TransportDynactin ComplexCell biologynervous system diseasesVesicular transport proteinDNA-Binding ProteinsBiochemistrynervous systembiology.proteinDynactinMicrotubule-Associated ProteinsNeurotrophinCell
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