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RabbitAnti-UNC93B Rabbit pAb  antibody (bs-8504R)
~~~促销,代码KXJ230206~~~
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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-8504R
英文名称 UNC93B Rabbit pAb
中文名称 内质网膜蛋白UNC93B抗体
别    名 IIAE1; UNC-93B; UNC93; UNC93B; Unc-93B1; UN93B_HUMAN; UNC93B1; hUNC93B1; UN93B_MOUSE;   
研究领域 细胞生物  免疫学  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 (predicted: Human, Mouse, Rat, )
产品应用 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 (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 66 kDa
细胞定位 细胞膜 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human UNC93B: 451-550/597 
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 UNC93B is a protein with similarity to the C. elegans unc93 protein. The Unc93 protein is involved in the regulation or coordination of muscle contraction in the worm. Defects in UNC93B are associated with susceptibility to herpes simplex encephalitis (HSE) which is a rare complication of human herpesvirus 1 (HHV-1) infection.

Function:
Plays an important role in innate and adaptive immunity by regulating nucleotide-sensing Toll-like receptor (TLR) signaling. Required for the transport of a subset of TLRs (including TLR3, TLR7 and TLR9) from the endoplasmic reticulum to endolysosomes where they can engage pathogen nucleotides and activate signaling cascades. May play a role in autoreactive B-cells removal.

Subunit:
Interacts with TLR3, TLR7, and TLR9 (probably via transmembrane domain).

Subcellular Location:
Membrane; Multi pass membrane protein.

Tissue Specificity:
Expressed in plasmocytoid dendritic cells (at protein level). Highly expressed in antigen-presenting cells. Expressed in heart, and at lower level in kidney. Expressed at low level in other tissues.

Post-translational modifications:
N-glycosylated (By similarity).

DISEASE:
Defects in UNC93B1 are associated with herpes simplex encephalitis type 1 (HSE1) [MIM:610551]. HSE is a rare complication of human herpesvirus 1 (HHV-1) infection, occurring in only a small minority of HHV-1 infected individuals. HSE is characterized by hemorrhagic necrosis of parts of the temporal and frontal lobes. Onset is over several days and involves fever, headache, seizures, stupor, and often coma, frequently with a fatal outcome. Note=Mutations in UNC93B1 resulting in autosomal recessive UNC93B1 deficieny predispose otherwise healthy individuals to isolated herpes simplex encephalitis due to impaired IFNs production. UNC93B1 deficieny, however, does not compromise immunity to most pathogens, unlike most known primary immunodeficiencies.

Similarity:
Belongs to the unc-93 family.

SWISS:
Q9H1C4

Gene ID:
81622

Database links:

Entrez Gene: 81622 Human

Entrez Gene: 54445 Mouse

Entrez Gene: 361689 Rat

Omim: 608204 Human

SwissProt: Q9H1C4 Human

SwissProt: Q8VCW4 Mouse



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