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货号： bs-11697R-Bio 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-11697R-Bio
- 英文名称
- Anti-HIPPI/Biotin
- 中文名称
- 生物素标记的舞蹈症相关相互作用蛋白1/雌激素相关受体样蛋白1抗体
- 别 名
- Dermal papilla derived protein 8; Dermal papilla-derived protein 8; DERP 8; DERP8; Esrrbl 1; Esrrbl1; ESRRBL1 protein; Estrogen related receptor beta like 1; Estrogen related receptor beta like protein 1; Estrogen-related receptor beta like 1; Estrogen-related receptor beta-like protein 1; FLJ10147; Hip1 Interacting Protein; HIP1 interacting protein HIPPI; Hip1 protein interactor; HIP1-interacting protein; HIPPI; Huntingtin interacting protein 1 interacting protein; Huntingtin interacting protein 1 protein interactor; Huntingtin-interacting protein-1 protein interactor; IFT 57; ift57; IFT57_HUMAN; Intraflagellar transport 57 homolog (Chlamydomonas); Intraflagellar transport 57 homolog; Intraflagellar transport protein 57 homolog; MHS4R2; Vestrogen related receptor beta like 1; Vestrogen-related receptor beta like 1; CDNA FLJ10147 fis clone HEMBA1003369.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 细胞生物 神经生物学 Alzheimers
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Cow, Horse, Rabbit,
- 产品应用
- WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 49kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human HIPPI (331-429aa)
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
Programmed neuronal cell death is a feature of neurodegenerative disorders such as Alzheimers and Huntingtons disease, which occur later in human life. Huntington’s disease at the molecular and cell level is characterized by polyglutamine expansion of the protein huntingtin (Htt) that leads to apoptotis-mediated neurodegenerative loss of medium spiny neurons throughout the striatum. Polyglutamine expansion reduces the level of association between Hip-1 and Htt, thereby increasing levels of free Hip-1 that then can be the candidate protein Hippi (Hip-1 protein interactor). The Hippi-Hip-1 heterodimer is a pro-apoptotic complex that recruits procaspase-8 and initiates caspase-8 activation, which may contribute to the neuronal cell death observed in individuals diagnosed with Huntington’s disease. The human hippi gene maps to chromosome 3q13.13 and encodes a 429 amino acid protein.
Function:
Required for the formation of cilia. Plays an indirect role in sonic hedgehog signaling, cilia being required for all activity of the hedgehog pathway (By similarity). Has pro-apoptotic function via its interaction with HIP1, leading to recruit caspase-8 (CASP8) and trigger apoptosis. Has the ability to bind DNA sequence motif 5-AAAGACATG-3 present in the promoter of caspase genes such as CASP1, CASP8 and CASP10, suggesting that it may act as a transcription regulator; however the relevance of such function remains unclear.
Subunit:
Component of IFT complex B composed of IFT88, IFT57, TRAF3IP1, IFT52, IFT27, HSPB11 and IFT20. Interacts with IFT20 and IFT88. Interacts with BLOC1S2 and RYBP. Interacts with HOMER1, possibly leading to prevent pro-apoptotic effects of IFT57 (By similarity). Interacts with HIP1. In normal conditions, it poorly interacts with HIP1, HIP1 being strongly associated with HTT. However, in mutant HTT proteins with a long poly-Gln region, interaction between HTT and HIP1 is inhibited, promoting the interaction between HIP1 and IFT57, leading to apoptosis. Interacts with BFAR. Interacts with chicken anemia virus protein apoptin.
Subcellular Location:
Cytoplasm, cytoskeleton, cilium basal body (By similarity). Note=Concentrates within the inner segment of cilia
Tissue Specificity:
Present in many tissues such as brain, thymus, lymph node, lung, liver, skin and kidney (at protein level).
Similarity:
Belongs to the IFT57 family.
Database links:
UniProtKB/Swiss-Prot: Q9NWB7.1
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.