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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
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 180 kb 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 13.7 kb and is expressed predominantly in adult and fetal brain whereas the smaller transcript of approximately 10.3 kb 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 #H7965-01F
ApplicationsSuitable for use in Dot Blot and Western Blot. Other applications not tested.
Recommended DilutionOptimal dilutions to be determined by the researcher.
Storage and StabilityMay be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, add sterile glycerol (40-50%), aliquot and store at -20°C. Aliquots are stable for at least 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Clone TypeMonoclonal
IsotypeIgG2b
Clone No8D100
HostMouse
SourceHuman
Concentration~0.1mg/ml
FormSupplied as a liquid in PBS, 7.4, 1% BSA and 0.05% sodium azide.
PurityPurified using Protein G column chromatography from culture supernatant of hybridoma cultured in a medium containing bovine IgG-depleted (~95%) fetal bovine serum.
ImmunogenPartial sequence of recombinant full-length protein to human HD
SpecificityRecognizes human HD.
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.
Alternate namesHD, HTT, IT15


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