神经性舞蹈病蛋白抗体
规格：1尘驳/1尘濒
英文名： Huntingtin
别名： 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.
分子量： 347kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human Huntingt
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞核 细胞浆
神经性舞蹈病蛋白抗体产物介绍：background: 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. 神经性舞蹈病蛋白抗体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神经性舞蹈病蛋白抗体 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. 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
神经性舞蹈病蛋白抗体产物应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：细胞生物 神经生物学 Alzheimer's
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid