Anti-Huntingtin/HTT Antibody Picoband® Fluoro488 Conjugated

The huntingtin gene, also called HTT or HD (Huntington disease) gene, is the IT15 (interesting transcript 15) gene which codes for a protein called the huntingtin protein. It is mapped to 4p16.3. The protein has no sequence homology with other proteins and is highly expressed in neurons and tests in humans and rodents. HTT upregulates the expression of Brain Derived Neurotrophic Factor (BDNF) at the transcription level, and this gene is required for normal development. The HTT protein is involved in vesicle trafficking as it interacts with HIP1, a clathrin-binding protein, to mediate endocytosis, the absorption of materials into a cell. HTT was also visualized as punctate staining likely to represent nerve endings. What's more, wildtype HTT may function in the nucleus in the assembly of nuclear matrix-bound protein complexes involved with transcriptional repression and RNA processing.

A synthetic peptide corresponding to a sequence at the C-terminus of human Huntingtin, different from the related mouse and rat sequences by two amino acids.

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. .

1. Kegel, K. B., Meloni, A. R., Yi, Y., Kim, Y. J., Doyle, E., Cuiffo, B. G., Sapp, E., Wang, Y., Qin, Z.-H., Chen, J. D., Nevins, J. R., Aronin, N., DiFiglia, M. Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription. J. Biol. Chem. 277: 7466-7476, 2002. 2. The Huntington's Disease Collaborative Research Group (March 1993) . A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72 (6) : 971–83. 3. Zuccato C, Ciammola A, Rigamonti D, Leavitt BR, Goffredo D, Conti L, MacDonald ME, Friedlander RM, Silani V, Hayden MR, Timmusk T, Sipione S, Cattaneo E (July 2001) . Loss of huntingtin-mediated BDNF gene transcription in Huntington's disease. Science 293 (5529) : 493–8.

Cytoplasm. Nucleus. 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.