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        Huntingtin Recombinant Rabbit mAb (bsm-54305R)  
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        產品編號 bsm-54305R
        英文名稱 Huntingtin Recombinant Rabbit mAb
        中文名稱 神經性舞蹈病蛋白重組兔單抗
        別    名 HD; HD protein; HD_HUMAN; HDH; HTT; Huntingtin; HUNTINGTON CHOREA; Huntington disease protein; Huntington's disease protein homolog; IT 15; IT15; OTTMUSP00000026909; ZHD; AI256365; C430023I11Rik.  
        研究領域 細胞生物  神經生物學  Alzheimer's  
        抗體來源 Rabbit
        克隆類型 Recombinant
        克 隆 號 1F10
        交叉反應 Human,Mouse,Rat
        產品應用 WB=1:500-2000,IHC-P=1:50-200,IHC-F=1:50-200,IF=1:50-200,ICC/IF=1:50-200
        not yet tested in other applications.
        optimal dilutions/concentrations should be determined by the end user.
        理論分子量 347 kDa
        檢測分子量
        細胞定位 細胞核 細胞漿 
        性    狀 Liquid
        濃    度 1mg/ml
        免 疫 原 A synthesized peptide derived from human Huntingtin: 1-47 
        亞    型 IgG
        純化方法 affinity purified by Protein A
        緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
        保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
        注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
        PubMed PubMed
        產品介紹 Huntingtin is a protein that contains a polyglutamine region. When the number of glutamine repeats exceeds 35, the gene encodes a version of Huntingtin that leads to Huntington’s disease (HD). When the polyglutamine stretch is mutated, Huntingtin acts within the nucleus to induce neurodegeneration by a cell-specific apoptotic mechanism. Loss of Huntingtin activity is unlikely to be the cause of HD, and it has been proposed that the expanded glutamine repeat region may induce an abnormal interaction between the mutant protein and other cellular proteins. Huntingtin interacts with a variety of proteins including HAP1, glyceraldehyde phosphate dehydrogenase (GAPDH) and HIP1.

        Function:
        May play a role in microtubule-mediated transport or vesicle function.

        Subunit:
        Binds SH3GLB1 (By similarity). Interacts through its N-terminus with PRPF40A. Interacts with PQBP1, SETD2 and SYVN. Interacts with PFN1.

        Subcellular Location:
        Cytoplasm. Nucleus. The mutant Huntingtin protein colocalizes with AKAP8L in the nuclear matrix of Huntington's disease neurons.

        Tissue Specificity:
        Expressed in the brain cortex (at protein level). 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.

        Post-translational modifications:
        Cleaved by apopain downstream of the polyglutamine stretch. The resulting N-terminal fragment is cytotoxic and provokes apoptosis.
        Forms with expanded polyglutamine expansion are specifically ubiquitinated by SYVN1, which promotes their proteasomal degradation.

        DISEASE:
        Defects in HTT 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:
        Belongs to the huntingtin family.
        Contains 10 HEAT repeats.

        SWISS:
        P42858

        Gene ID:
        3064

        Database links:

        Entrez Gene: 3064 Human

        Entrez Gene: 15194 Mouse

        Entrez Gene: 29424 Rat

        Omim: 143100 Human

        Omim: 613004 Human

        SwissProt: P42858 Human

        SwissProt: P42859 Mouse

        SwissProt: P51111 Rat

        Unigene: 518450 Human

        Unigene: 209071 Mouse

        Unigene: 482929 Mouse

        Unigene: 11193 Rat



        產品圖片
        25 ug total protein per lane of various lysates (see on figure) probed with Huntingtin monoclonal antibody, unconjugated (bsm-54305R) at 1:2000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
        Paraformaldehyde-fixed, paraffin embedded Human Cerebellum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
        Paraformaldehyde-fixed, paraffin embedded Rat Cerebellum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
        Paraformaldehyde-fixed, paraffin embedded Human Testicles; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
        Paraformaldehyde-fixed, paraffin embedded Rat Testicles; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
        Paraformaldehyde-fixed, paraffin embedded Human Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
        Paraformaldehyde-fixed, paraffin embedded Rat Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
        Paraformaldehyde-fixed, paraffin embedded Mouse Cerebrum; Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15 min; Antibody incubation with Huntingtin Monoclonal Antibody, Unconjugated(bsm-54305R) at 1:200 overnight at 4°C, followed by conjugation to the bs-0295G-HRP and DAB (C-0010) staining.
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