Human Huntingtin ELISA Kit (Colorimetric)

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).

This product is for research use only and is not approved for use in humans or in clinical diagnosis. ELISA Kits are guaranteed for 6 months from date of receipt.

Array NBP3-39911

• Huntingtin Antibodies
• Huntingtin ELISA Kits
• Huntingtin Proteins