Huntingtin Antibody, mAb, Mouse

Huntingtons disease (HD) is a neurodegenerative disorder caused by an expanding polyglutamine repeat in the huntingtin gene. HD is a mid-life onset autosomal dominant neurodegenerative disease that is characterized by psychiatric disorders, dementia, and involuntary movements (chorea), leading to death in 10 to 20 years. The HD gene product is widely expressed in human tissues, with the highest level of expression in the brain. The huntingtin gene product is expressed at similar levels in patients and controls, which suggests that the expansion of the polyglutamine repeat induces a toxic gain of function perhaps through interactions with other cellular proteins. Using a yeast two-hybrid system, researchers have identified a protein, HAP1 (huntingtin associated protein 1), that associates with huntingtin protein. The in vitro data suggest that the association between HAP1 and huntingtin is enhanced by increasing the length of the glutamine repeat.