抗Huntingtin抗体 | Anti-Huntingtin Antibody

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Western Blot: Huntingtin Antibody [NBP1-44265] - Lane 1: mouse brain extract. Lane 2: mouse brain extract with blocking peptide. Load: 10 ug per lane. Primary antibody: Huntingtin antibody at 0.1ug/mL for overnight at 4C. Secondary antibody: IRDye800 rabbit secondary antibody at 1:10,000 for 45 min at RT. Block: 5% BLOTTO overnight at 4C. Predicted/Observed size: 350 kDa for Huntington. Other band(s): Huntingtin splice variants and isoforms.

Western Blot: Huntingtin Antibody [NBP1-44265] - Western blot analysis on mouse brain extract using 0.1ug/mL of NBP1-44265 (lane 1) and 0.1ug/mL of NBP1-44265 combined with 1ug/mL of blocking peptide (lane 2).

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Background

Huntingtin (also known as Huntington's disease protein, Htt and HD protein) is the protein product of a disease gene linked to Huntington's disease, a neuro-degenerative disorder characterized by loss of striatal neurons. This may be caused by an expanded, unstable trinucleotide repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product (see partial protein sequence below). The huntingtin gene locus is large, spanning 180 kb and consisting of 67 exons. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The genetic defect leading to Huntington's disease may not necessarily eliminate transcription, but may confer a new property on the mRNA or alter the function of the protein. One candidate is the huntingtin-associated protein-1, highly expressed in brain, which has increased affinity for huntingtin protein with expanded polyglutamine repeats. Normal huntingtin protein shows a cytoplasmic localization. This protein is 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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