货号：MAB2174

品牌：Merck Millipore

规格：

目录价：￥3609.00

市场价格：￥3067.65

会员价格：￥2887.20

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549 | MAB2174 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Huntingtin protein residues 549-679. Huntingtin localization by immunocytochemistry is primarily neuronal. Glia contain very little huntingtin; huntingtin is abundant in perikarya and dendrites of cortical pyramidal cells and in Purkinje cells, and striatal interneurons. Clone mHD549 has been tested in human, rat, and monkey. View All» Epitope: a.a. 549-679 View All» Immunogen: Huntingtin fusion protein 549-679, fused to Glutathione S-tranferase. View All» Clone: mHD549 View All» Isotype: Ig View All» Species Reactivity:

• Monkey

• Rat
  View All» Application Notes: Immunohistochemistry on 3%/0.1% glutaraldehyde paraformaldehyde fixed, frozen tissue, using enzymatic detection. Suggested starting dilution 1:1,000. See Gutekunst et al, 1998. Does not work on paraffin embedded tissues. For EM, see Gutekunst, 1995 PNAS, 92:8710-8714. Immunocytochemistry. Immunoblotting: SDS/PAGE, PVDF membranes, 50μg/lane total protein; 7.5% PAGE. Detects 340kDa ITI5 open reading frame in lymphoblasts from human controls; Additional protein bands are detected in monkey (130-140kDa). Western blots {Gutekunst, et al, 1995}. Normal (340kDa) and mutant (> 340kDa) bands can be detected in lymphoblast lysates from homozygote or heterozygote HD patients via SDS/4% PAGE. Abundant in rat tetis (45-50kDa) (Gutekunst, 1995). Optimal working dilutions and protocols must be determined by end user. View All» Applications Not Recommended: Immunohistochemistry (Paraffin) View All» Presentation: Ascites fluid. Liquid with 0.1% Sodium azide. View All» Storage Conditions: Maintain at -20°C in undiluted aliquots for up to 12 months. Avoid repeated freeze/thaw cycles. 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:
    • Western Blotting

    • Immunocytochemistry

    • Immunohistochemistry
      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. 549-679, clone mHD549 | MAB2174 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µL View All» Format: Ascites View All» Host: Rat View All»
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