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You are here:Home » Antibodies » Abs to Disease Markers » Anti -HTT (Huntington Disease Protein, HD Protein, HD, IT15)

Anti -HTT (Huntington Disease Protein, HD Protein, HD, IT15)

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Specifications

Clone Host Grade Applications
Monoclonal Mouse Affinity Purified E B IH
Huntingtin is a disease gene linked to Huntington's disease, a neurodegenerative disorder characterized by loss of striatal neurons. This is thought to be caused by an expanded, unstable trinucleotide repeat in the huntingtin gene, which translates as a polyglutamine repeat in the protein product. A fairly broad range in the number of trinucleotide repeats has been identified in normal controls, and repeat numbers in excess of 40 have been described as pathological. The huntingtin locus is large, spanning 180kb and consisting of 67 exons. The huntingtin gene is widely expressed and is required for normal development. It is expressed as 2 alternatively polyadenylated forms displaying different relative abundance in various fetal and adult tissues. The larger transcript is approximately 13.7kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3kb is more widely expressed. 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. This gene contains an upstream open reading frame in the 5' UTR that inhibits expression of the huntingtin gene product through translational repression.
Catalog #H7965-02D
ApplicationsSuitable for use in ELISA, Western Blot and Immunohistochemistry. Other applications not tested.
Recommended DilutionImmunohistochemistry (Formalin fixed paraffin embedded): 5ug/ml
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 at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypeMonoclonal
IsotypeIgG2a,k
Clone No3F1
HostMouse
SourceHuman
Concentration~0.5mg/ml
FormSupplied as a liquid in PBS, pH 7.2.
PurityPurified by Protein A affinity chromatography.
ImmunogenRecombinant corresponding to aa81-191 from human HD with GST tag. MW of the GST tag alone is 26kD.
SpecificityRecognizes human Huntingtin.
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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