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品种反应性 主要应用 宿主 格式 抗体类型 H WB, ELISA, ICC, IHC M Ascites Monoclonal Antibody 描述 产品目录编号 MAB5492 品牌系列 Chemicon®
商名 - Chemicon
描述 Anti-Huntingtin Antibody, a.a. 1-82 产品信息 格式 Ascites 控制 - Normal human cerebral cortex lysate, Mouse brain cortex samples from HD or wild type mice
演示 Ascites fluid containing no preservatives. 应用 应用 Anti-Huntingtin Antibody, a.a. 1-82 is an antibody against Huntingtin for use in ELISA, IC, IH & WB. 主要应用 - Western Blotting
- ELISA
- Immunocytochemistry
- Immunohistochemistry
应用说明 Western blot: 1:500-1:5,000
Immunocytochemistry (1): 1:500-1:5,000
Immunohistochemistry (1,2): 1:500-1:5,000
ELISA: 1:500-1:5,000
Optimal working dilutions must be determined by end user.生物信息 免疫原品种 Recombinant human huntingtin, amino acids 1-82. 表位 a.a. 1-82 克隆 2B4 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Mouse 特异性 Reacts huntingtin protein, amino acids 1-82. The antibody recognizes wild type and mutant huntingtin. 同种型 IgG1 品种反应性 Human 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
纯化方法 Unpurified 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 for 1 year at -20°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. 包装信息 数量 100 µL
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