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Mouse Anti-RBCK1  antibody (bsm-51685M)
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说明书: 50ul  100ul  
50ul/1180.00元
100ul/1980.00元
大包装/询价

产品编号 bsm-51685M
英文名称 RBCK1
中文名称 乙型肝炎病毒相关因子4单克隆抗体
别    名 C20orf18; HOIL1_HUMAN; RanBP-type and C3HC4-type zinc finger-containing protein 1; HBV-associated factor 4; Heme-oxidized IRP2 ubiquitin ligase 1; HOIL-1; Hepatitis B virus X-associated protein 4; RING finger protein 54; RING-type E3 ubiquitin transferase HOIL-1; Ubiquitin-conjugating enzyme 7-interacting protein 3; RNF54; UBCE7IP3; XAP3; XAP4.  
研究领域 转录调节因子  细菌及病毒  锌指蛋白  表观遗传学  泛素  
抗体来源 Mouse
克隆类型 Monoclonal
克 隆 号 R5E2
交叉反应 Human
产品应用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 56kDa
细胞定位 细胞核 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 Recombinant human RBCK1 (UBCE7IP3). 
亚    型 IgG1,k
纯化方法 affinity purified by Protein G
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C 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 The protein encoded by this gene is similar to mouse UIP28/UbcM4 interacting protein. Alternative splicing has been observed at this locus, resulting in distinct isoforms. [provided by RefSeq, Jul 2008]

Function:
Acts as an E3 ubiquitin-protein ligase, or as part of an E3 complex, which accepts ubiquitin from specific E2 ubiquitin-conjugating enzymes, such as UBE2L3/UBCM4, and then transfers it to substrates. Functions as an E3 ligase for oxidized IREB2 and both heme and oxygen are necessary for IREB2 ubiquitination. Promotes ubiquitination of TAB2 and IRF3 and their degradation by the proteoasome. Component of the LUBAC complex which conjugates linear polyubiquitin chains in a head-to-tail manner to substrates. LUBAC conjugates linear polyubiquitin to IKBKG at 'Lys-285' and 'Lys-309' and is involved in activation of the canonical NF-kappa-B and the JNK signaling pathways. LUBAC is proposed to be recruited to the TNF-R1 signaling complex (TNF-RSC) following polyubiquitination of TNF-RSC components by BIRC2 and/or BIRC3 and to conjugate linear polyubiquitin to IKBKG and possibly other components contributing to the stability of the complex. Binds polyubiquitin of different linkage types.

Subcellular Location:
Forms homodimers in vitro (By similarity). Component of the LUBAC complex (linear ubiquitin chain assembly complex) which consists of SHARPIN, RBCK1 and RNF31. LUBAC has a MW of approximative 600 kDa suggesting a heteromultimeric assembly of its subunits. Interacts with beta-I-type (PRKCB1) and zeta-type protein kinase C (PRKCZ) and with UBE2L3. Interacts with PRKCH. Interacts with the HBV pX/HBx protein, which is required to activate transcription of the viral genome. Isoform 1 and isoform 2 interact with IREB2 only in iron-rich conditions. Associates with the TNF-R1 signaling complex (TNF-RSC) in a stimulation-dependent manner. Interacts with EYA1, TAB2, TAB3, MAP3K7 TRAF6 and RIPK1. Interacts with IRF3.

Post-translational modifications:
Auto-ubiquitinated. Auto-ubiquitination leads to degradation by the proteasome. Phosphorylated. In vitro, phosphorylation inhibits auto-ubiquitination activity.

DISEASE:
The disease is caused by mutations affecting the gene represented in this entry.
Disease description:A disease characterized by polyglucosan storage myopathy associated with early-onset progressive muscle weakness and progressive dilated cardiomyopathy, which may necessitate cardiac transplant in severe cases. Some patients present with severe immunodeficiency, invasive bacterial infections and chronic autoinflammation.

Similarity:
Contains 1 B box-type zinc finger.
Contains 1 RanBP2-type zinc finger.
Contains 1 RING-type zinc finger.
Contains 1 ubiquitin-like domain.

SWISS:
Q9BYM8

Gene ID:
10616

Database links:

Entrez Gene: 10616 Human

SwissProt: Q9BYM8 Human



产品图片
Sample:
Lane 1: Jurkat cell lysates
Lane 2: 293 cell lysates
Lane 3: A375 cell lysates
Primary: Anti-RBCK1 (bsm-51685M) at 1/2000 dilution
Secondary: IRDye800CW Goat Anti-Mouse IgG at 1/20000 dilution
Predicted band size: 56 kD
Observed band size: 54 kD
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