磷酸化神经性舞蹈病蛋白抗体
规格：1mg/1ml
英文名： phospho-Huntingtin (Ser421)
别名： Huntingtin (phospho S421); p-Huntingtin (phospho S421); phospho-Huntingtin (Ser419); Huntingtin (phospho Ser419); Huntingtin (phospho S419); AI256365; C430023I11Rik; HD; HD protein; HD_HUMAN; HDH; HTT
分子量： 348kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthesised phosphopeptide derived from human
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse, Sheep,
细胞定位：细胞核 细胞浆
磷酸化神经性舞蹈病蛋白抗体产品介绍：background: 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 ***** tissues. The larger transcript is approximately 13.7 kb and is expressed predominantly in ***** 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. [provided by RefSeq, Jul 2008] Function: May play a role in microtubule-mediated transport or vesicle function.磷酸化神经性舞蹈病蛋白抗体 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: modificationsCleaved 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磷酸化神经性舞蹈病蛋白抗体 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: 磷酸化神经性舞蹈病蛋白抗体This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品应用：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
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid