Species: Hu, Mu
Applications: WB, IHC, IHC-Fr
Host: Mouse Monoclonal
Species: Hu, Mu, Rt
Applications: WB, Flow, ICC/IF, IHC, IHC-P
Host: Rabbit Monoclonal
Hu, Mu, RtApplications:
WB, ELISA, ICC/IF, IHCHost:
WB, ELISA, PA, PAGE, AP
Applications: RNAi, RNAi SP
Huntingtin protein (Htt) is a 348 kDa protein product of Huntington's disease (HD, IT15) gene. Huntington's disease is a neurodegenerative disorder caused by a mutation on the HD gene, producing a polyglutamin (polyQ) expansion on the N-terminus of Htt. The mutant Htt leads to cytotoxicity in the striatal neuron (1). This cytotoxicity is modulated by proteolytic cleavage with caspases and calpains, producing N-terminal polyQ fragments. A phosphorylation of Htt may also regulate the cleavage and cytotoxicity of mutant Htt (2). The wild-type Htt up-regulates transcription of brain-derived neurotrophic factor (BDNF), a pro-survival factor for striatal neurons. This suggests restoring wild-type Htt level and increasing BDNF expression level can be therapeutic treatment for Huntington's disease (3). Huntingtin is also cleaved by apopain, a cysteine protease product of death-gene for human, suggesting HD might be a disorder of inappropriate apoptosis (4).
Bioinformatics Tool for Huntingtin
Discover related pathways, diseases and genes to Huntingtin. Need help? Read the Bioinformatics Tool Guide
for instructions on using this tool.