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RabbitAnti-HIP1 Rabbit pAb  antibody (bs-11701R)
~~~促销,代码KXJ230206~~~
订购热线:400-901-9800
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-11701R
英文名称 HIP1 Rabbit pAb
中文名称 亨廷顿(舞蹈症)相互作用蛋白1抗体
别    名 Huntingtin Interacting Protein HIP1; HIP I; HIP-1; HIP-I; hip1; HIP1/PDGFRB fusion gene; HIP1/PDGFRB fusion gene, included; HIP1_HUMAN; HIPI; Huntingtin interacting protein 1; Huntingtin-interacting protein 1; Huntingtin-interacting protein I; ILWEQ; KIAA4113; MGC126506; MGC27616; mKIAA4113.  
研究领域 细胞生物  神经生物学  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 (predicted: Human, Mouse, Rat, Chicken, Dog, Horse, Rabbit, )
产品应用 WB=1:500-2000 ELISA=1:5000-10000 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.
理论分子量 116 kDa
检测分子量
细胞定位 细胞核 细胞浆 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HIP1: 401-500/1037 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 Huntington disease is associated with the expansion of a polyglutamine tract, greater than 35 repeats, in the HD gene product huntingtin. HIP1 (huntingtin-interacting protein 1), a membrane-associated protein, binds specifically to the N-terminus of human huntingtin. HIP1 is ubiquitously expressed in different brain regions at low levels, and exhibits nearly identical subcellular fractionation as huntingtin. The huntingtin-HIP1 interaction is restricted to the brain and is inversely correlated to the polyglutamine length in the huntingtin, suggesting that loss of normal huntingtin-HIP1 interaction may compromise the membrane-cytoskeletal integrity in the brain. HIP1 contains an endocytic multidomain protein with a C-terminal Actin-binding domain, a central coiled-coil forming region and an N-terminal ENTH domain. HIP1 may be involved in vesicle trafficking; the structural integrity of HIP1 is crucial for maintenance of normal vesicle size in vivo. HIP12 is a non-proapoptotic member of the HIP gene family that is expressed in the brain and shares a similar subcellular distribution pattern with HIP1. However, HIP12 differs from HIP1 in its pattern of expression at both the mRNA and protein level. HIP12 does not directly interact with huntingtin but can interact with HIP1.

Function:
Plays a role in clathrin-mediated endocytosis and trafficking. Involved in regulating AMPA receptor trafficking in the central nervous system in an NMDA-dependent manner. Enhances androgen receptor (AR)-mediated transcription. May act as a proapoptotic protein that induces cell death by acting through the intrinsic apoptosis pathway. Binds 3-phosphoinositides (via ENTH domain). May act through the ENTH domain to promote cell survival by stabilizing receptor tyrosine kinases following ligand-induced endocytosis. May play a functional role in the cell filament networks. May be required for differentiation, proliferation, and/or survival of somatic and germline progenitors.

Subunit:
Homodimer. Binds actin. Binds HTT (via N-terminus). This interaction is restricted to the brain. Binds to IFT57. In normal conditions, it poorly interacts with IFT57, 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. Interacts with CLTB (via N-terminus). Interacts (via coiled coil domain) with AR. Interacts with AP2A1, AP2A2, CLTC and HIP1R. Interacts with GRIA1, GRIN2A AND GRIN2B.

Subcellular Location:
Cytoplasm. Nucleus. Endomembrane system. Cytoplasmic vesicle > clathrin-coated vesicle membrane. Shuttles between cytoplasm and nucleus. Nuclear translocation can be induced by AR.

Tissue Specificity:
Ubiquitously expressed with the highest level in brain. Expression is up-regulated in prostate and colon cancer.

DISEASE:
Note=A chromosomal aberration involving HIP1 is found in a form of chronic myelomonocytic leukemia (CMML). Translocation t(5;7)(q33;q11.2) with PDGFRB. The chimeric HIP1-PDGFRB transcript results from an in-frame fusion of the two genes. The reciprocal PDGFRB-HIP1 transcript is not expressed.

Similarity:
Belongs to the SLA2 family.
Contains 1 ENTH (epsin N-terminal homology) domain.
Contains 1 I/LWEQ domain.

SWISS:
O00291

Gene ID:
3092

Database links:

Entrez Gene: 3092 Human

Entrez Gene: 215114 Mouse

Entrez Gene: 192154 Rat

Omim: 601767 Human

SwissProt: O00291 Human

SwissProt: Q8VD75 Mouse

Unigene: 329266 Human

Unigene: 619089 Human

Unigene: 280805 Mouse

Unigene: 836 Rat



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