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You are here:Home » Antibodies » Abs to Disease Markers » Anti -Huntingtin Protein (Huntington Disease)

Anti -Huntingtin Protein (Huntington Disease)

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Specifications

Clone Host Grade Applications
Monoclonal Mouse Affinity Purified B IH
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.
Huntingtin 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.
Catalog #H7965-01C
ApplicationsSuitable for use in Western Blot and Immunohistochemistry in lipid metabolic research. Other applications not tested.
Recommended DilutionsWestern Blot: 1:1000. Detects a 350kD band, also detects smaller degradation products of huntingtin in some tissue lysates.
Immunohistochemistry: 1:1000
Optimal dilutions to be determined by the researcher.
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypeMonoclonal
IsotypeIgG1
Clone No8A181
HostMouse
Concentration~1mg/ml
FormSupplied as a liquid in PBS, pH 7.4, 0.05% sodium azide.
PurityPurified by immunoaffinity chromatography.
ImmunogenSynthetic peptides corresponding to aa1-225 of the N-terminal Huntingtin Protein.
SpecificityRecognizes Hungtingtin. Species Crossreactivity: human and mouse
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.


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