Huntingtin Polyclonal Antibody Sizes: 50µl, 100µl Catalogue Numbers: BS76983-50, BS76983-100 Product: 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2 Swiss-Prot: P42858 Host: Rabbit Reactivity: Human, Mouse, Rat Applications: WB, IHC, IF/ICC All Applications: WB,1:500 - 1:2000 | IHC,1:50 - 1:200 | IF/ICC,1:50 - 1:200 Background: 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 of trinucleotide repeats (9-35) 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. Purification and Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE). Storage and Stability: Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles. Specificity: Unmodification Bioworld Molecular Weight: 347kDa Note: For research use only, not for use in diagnostic procedure. Extra Notes: Western blot analysis of extracts of various cell lines, using Huntingtin antibody at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG at 1:10000 dilution. Lysates/proteins: 25ug per Lane. Blocking buffer: 3% non-fat dry milk in TBST. Detection: ECL Basic Kit. Exposure time: 10s., Immunohistochemistry of paraffin-embedded rat brain using Huntingtin Rabbit pAb at dilution of 1:100. Perform microwave antigen retrieval with 10 mM Tris/EDTA buffer pH 9.0 before commencing with IHC staining protocol., Immunofluorescence analysis of U-251 MG cells using Huntingtin Rabbit pAb at dilution of 1:100. Blue: DAPI for nuclear staining. Alternative Name: HD; IT15; HTT; Huntingtin Immunogen: Recombinant fusion protein of human Huntingtin(NP_002102.4). Conjugate: Unconjugated Modification: Unmodified