Huntingtin Protein (Huntington Disease)

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.\nHuntingtin is a 350kD protein that is mutated in Huntington's disease (HD). The normal function of huntingtin still remains uncertain but it has been suggested that it performs a necessary housekeeping function. The abnormal form of huntingtin aggregates in vitro and forms neuronal intranuclear and cytoplasmic inclusions in HD patients. \n\nApplications: \nSuitable for use in Western Blot and Immunohistochemistry in lipid metabolic research. Other applications not tested.\n\nRecommended Dilutions:\nWestern Blot: (same/more than)1:1000. Detects a 350kD band, also detects smaller degradation products of huntingtin in some tissue lysates.\nImmunohistochemistry: (same/more than)1:1000\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

H7965-01C

Size

100ug

Applications

IHC, WB

Hosts

Mouse

Reactivities

Hum, Mouse

Form

Supplied as a liquid in PBS, pH 7.4, 0.05% sodium azide.

P Type

Mab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG1

References

1. Khoshnan A, et al. Effects of intracellular expression of anti-huntingtin antibodies of various specificities on mutant huntingtin aggregation and toxicity. Proc. Natl. Acad Sci. U. S. A. Jan 2002; 99(2): 1002-1007. 2. Ko J, et al. New anti-huntingtin monoclonal antibodies: implications for huntingtin conformation and its binding proteins. Brain Res. Bull. Oct-Nov 2001; 56(3-4): 319-329.

Additional Info

Recognizes Hungtingtin. Species Crossreactivity: human and mouse