|Application ||WB, IHC|
|Reactivity||Human, Mouse, Rat|
|Calculated MW||347603 Da|
|Other Names||Huntingtin, Huntington disease protein, HD protein, HTT, HD, IT15|
|Target/Specificity||A synthetic peptide corresponding to residues specific to the apopain cleavage site of human huntingtin protein was used as an immunogen|
|Format||50 mM Tris-Glycine (pH 7.4), 0.15 M NaCl, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||Huntingtin (HTT) Antibody is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||May play a role in microtubule-mediated transport or vesicle function.|
|Cellular Location||Cytoplasm. Nucleus. Note=The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington disease neurons. Shuttles between cytoplasm and nucleus in a Ran GTPase-independent manner.|
|Tissue Location||Expressed in the brain cortex (at protein level). Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation.|
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Provided below are standard protocols that you may find useful for product applications.
Huntingtin protein (Htt) is a 348 kDa protein product of Huntingtons disease (HD, IT15) gene. Huntingtons 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 Huntingtons 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).
1. Mende-Mueller LM, et al. J Neuro 21:1830-1837, 2001.
2. Schilling B, et al. J Biol Chem 281:23686-23697, 2006.
3. Zuccato C, et al. Science 293:445-446, 2001.
4. Goldberg YP, et al Nat Genet 13:442-449, 1996.
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