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HIPPI Rabbit pAb (bs-11697R)  
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產(chǎn)品編號 bs-11697R
英文名稱 HIPPI Rabbit pAb
中文名稱 舞蹈癥相關(guān)相互作用蛋白1/雌激素相關(guān)受體樣蛋白1抗體
別    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.  
研究領(lǐng)域 細胞生物  神經(jīng)生物學  Alzheimer's  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應(yīng) Human,Mouse,Rat (predicted: Rabbit,Cow,Dog,Horse)
產(chǎn)品應(yīng)用 WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 49 kDa
檢測分子量
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HIPPI: 331-429/429 
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(chǎn)品介紹 Programmed neuronal cell death is a feature of neurodegenerative disorders such as Alzheimer's and Huntington's 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.

SWISS:
Q9NWB7

Gene ID:
55081

Database links:

Entrez Gene: 55081 Human

Entrez Gene: 73916 Mouse

Omim: 606621 Human

SwissProt: Q9NWB7 Human

SwissProt: Q8BXG3 Mouse

Unigene: 412196 Human

Unigene: 241276 Mouse

Unigene: 404371 Mouse



產(chǎn)品圖片
Sample: Lane 1: Mouse Cerebrum tissue lysates Lane 2: Rat Cerebrum tissue lysates Lane 3: Human 293T cell lysates Lane 4: Human HepG2 cell lysates Primary: Anti-HIPPI (bs-11697R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 49 kDa Observed band size: 50 kDa
Sample: A431(Human) Cell Lysate at 30 ug Primary: Anti- HIPPI (bs-11697R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 49 kD Observed band size: 52 kD
Paraformaldehyde-fixed, paraffin embedded (rat brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (HIPPI) Polyclonal Antibody, Unconjugated (bs-11697R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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