货号：MAB2168

品牌：Merck Millipore

规格：100UL

目录价：￥7049.00

市场价格：￥5991.65

会员价格：￥5639.20

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Huntingtin Protein Antibody, a.a. 2146-2541, clone HU-2E8 | MAB2168 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Reacts with Huntingtin protein from human and monkey. Weak to no reactivity with mouse. Has not been tested on other species. View All» Epitope: a.a. 2146-2541 View All» Immunogen: Human huntingtin fragment from aa 2146 to 2541 as a fusion protein. View All» Clone: HU-2E8 View All» Isotype: IgG1 View All» Species Reactivity:

• Human

• Monkey
  View All» Application Notes: ELISA: 1:500-1:5,000 Western blot: 1:500-1:5,000 Immunohistochemistry on frozen and microwave oven treated paraffin sections (human tissue): 1:500-1:5,000 Immunocytochemistry on transfected cells: 1:500-1:5,000 Optimal working dilutions must be determined by the end user. View All» Presentation: Ascites fluid. Liquid, does not contain any preservative. View All» Storage Conditions: Maintain at -20°C in undiluted aliquots up to 12 months after date of receipt. Avoid repeated freeze/thaw cycles. During shipment, small volumes of antibody will occasionally become entrapped in the seal of the product vial. For antibodies with volumes of 200 μl or less, we recommend gently tapping the vial on a hard surface or briefly centrifuging the vial in a tabletop centrifuge to dislodge any liquid in the container's cap. View All» UniProt Number: P42858 View All» Entrez Gene Number: NM_002111.6 View All» Gene Symbol:
  • HTT

  • IT15

  • SLC6A4

  • Huntingtin

  • OCD1

  • HD
    View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
    • ELISA

    • Western Blotting

    • Immunocytochemistry

    • Immunohistochemistry (Paraffin)
      View All» Entrez Gene Summary: 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. View All» UniProt Summary: FUNCTION: SwissProt: P42858 # May play a role in microtubule-mediated transport or vesicle function.SIZE: 3144 amino acids; 347860 Da SUBUNIT: Binds SH3GLB1 (By similarity). Interacts through its N- terminus with FNBP3. Interacts with PQBP1, SETD2 and SYVN.SUBCELLULAR LOCATION: Cytoplasm. Nucleus.TISSUE SPECIFICITY: Widely expressed with the highest level of expression in the brain (nerve fibers, varicosities, and nerve endings). In the brain, the regions where it can be mainly found are the cerebellar cortex, the neocortex, the striatum, and the hippocampal formation.PTM: Cleaved by apopain downstream of the polyglutamine stretch. The resulting amino-terminal fragment is cytotoxic and provokes apoptosis. & Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation.DISEASE: SwissProt: P42858 # Defects in HD are the cause of Huntington disease (HD) [MIM:143100]. HD is an autosomal dominant neurodegenerative disorder characterized by involuntary movements (chorea), general motor impairment, psychiatric disorders and dementia. Onset of the disease occurs usually in the third or fourth decade of life and symptoms progressively worsen leading to death in 10 to 20 years. Onset and clinical course depend on the degree of poly-Gln repeat expansion, longer expansions resulting in earlier onset and more severe clinical manifestations. HD affects 1 in 10,000 individuals of European origin. Neuropathology of Huntington disease displays a distinctive pattern with loss of neurons, especially in the caudate and putamen (striatum).SIMILARITY: SwissProt: P42858 ## Belongs to the hungtintin family. & Contains 10 HEAT repeats. View All» Brand Family: Chemicon View All» Product Name: Anti-Huntingtin Protein Antibody, a.a. 2146-2541, clone HU-2E8 | MAB2168 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Ascites View All» Host: Mouse View All»
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