merck millipore,默克密理愽
  • merck millipore,默克密理博,MAB2174,Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549

  • merck millipore,默克密理博,MAB2174,Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549

    产品名称：Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549
    产品型号：MAB2174
    This Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549 is validated for use in WB, IC, IH for the detection of Huntingtin Protein.

    merck millipore,默克密理博,MAB2174,Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549

  • 产品介绍
  • merck millipore,默克密理博,MAB2174,Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549

    重要规格表

    品种反应性 主要应用 宿主 格式 抗体类型
    R, MkIHC, WB, ICCRAscitesMonoclonal Antibody
    描述
    产品目录编号 MAB2174
    品牌系列 Chemicon®
    商名
    • Chemicon
    描述 Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549
    产品信息
    格式 Ascites
    演示 Ascites fluid. Liquid with 0.1% Sodium azide.
    应用
    应用 This Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549 is validated for use in WB, IC, IH for the detection of Huntingtin Protein.
    主要应用
    • Immunohistochemistry
    • Western Blotting
    • Immunocytochemistry
    不建议的应用
    • Immunohistochemistry (Paraffin)
    应用说明 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.
    生物信息
    免疫原品种 Huntingtin fusion protein 549-679, fused to Glutathione S-tranferase.
    表位 a.a. 549-679
    克隆 mHD549
    宿主 Rat
    特异性 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.
    同种型 Ig
    品种反应性 RatMonkey
    抗体类型 Monoclonal Antibody
    Entrez基因编号
    • NM_002111.6
    Entrez基因汇总 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.
    基因符号
    • HD
    • HTT
    • IT15
    • SLC6A4
    • Huntingtin
    • OCD1
    UniProt编号
    • P42858
    UniProt汇总 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.
    产品使用声明
    使用声明
    • 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.
    储存和货运信息
    存储条件 Maintain at -20°C in undiluted aliquots for up to 12 months. Avoid repeated freeze/thaw cycles.
    包装信息
    数量 100 µL

    merck millipore,默克密理博,MAB2174,Anti-Huntingtin Protein Antibody, a.a. 549-679, clone mHD549

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