Huntingtin Protein

• 

  Subject Areas on Research

  • Abnormal degradation of the neuronal stress-protective transcription factor HSF1 in Huntington's disease. 
  • Activated microglia proliferate at neurites of mutant huntingtin-expressing neurons. 
  • Altered diffusion tensor imaging measurements in aged transgenic Huntington disease rats. 
  • CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease. 
  • Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract. 
  • Dopamine D2 receptor relies upon PPM/PP2C protein phosphatases to dephosphorylate huntingtin protein. 
  • Dopamine enhances motor and neuropathological consequences of polyglutamine expanded huntingtin. 
  • Dysregulation of C/EBPalpha by mutant Huntingtin causes the urea cycle deficiency in Huntington's disease. 
  • Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. 
  • Experimental models for identifying modifiers of polyglutamine-induced aggregation and neurodegeneration. 
  • Focal expression of mutant huntingtin in the songbird basal ganglia disrupts cortico-basal ganglia networks and vocal sequences. 
  • Folding Landscape of Mutant Huntingtin Exon1: Diffusible Multimers, Oligomers and Fibrils, and No Detectable Monomer. 
  • Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. 
  • Huntingtin is required for normal excitatory synapse development in cortical and striatal circuits. 
  • Hyperactivity and cortical disinhibition in mice with restricted expression of mutant huntingtin to parvalbumin-positive cells. 
  • IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome. 
  • IKKβ and mutant huntingtin interactions regulate the expression of IL-34: implications for microglial-mediated neurodegeneration in HD. 
  • Identification and evaluation of small molecule pan-caspase inhibitors in Huntington's disease models. 
  • In vitro effects of polyglutamine tracts on Ca2+-dependent depolarization of rat and human mitochondria: relevance to Huntington's disease. 
  • Inhibitors of protein disulfide isomerase suppress apoptosis induced by misfolded proteins. 
  • Intrabodies binding the proline-rich domains of mutant huntingtin increase its turnover and reduce neurotoxicity. 
  • Morphology, Migration, and Transcriptome Analysis of Schwann Cell Culture on Butterfly Wings with Different Surface Architectures. 
  • Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. 
  • PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically. 
  • Perturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1. 
  • Selective inhibitors of death in mutant huntingtin cells. 
  • Small molecule-induced oxidation of protein disulfide isomerase is neuroprotective. 
  • TR-FRET-based duplex immunoassay reveals an inverse correlation of soluble and aggregated mutant huntingtin in huntington's disease. 
  • Targeting mutant huntingtin for the development of disease-modifying therapy. 
  • The many faces of autophagy dysfunction in Huntington's disease: from mechanism to therapy. 
  • The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's disease protein, huntingtin.