Home
>
Product
>
Antibody
>
Rabbit Anti-RBCK1/HBV associated factor 4/FITC Conjugated antibody
background:
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.
Database links:
Entrez Gene: 10616 Human
Entrez Gene: 24105 Mouse
Omim: 610924 Human
SwissProt: Q9BYM8 Human
SwissProt: Q9WUB0 Mouse
Unigene: 247280 Human
Unigene: 182145 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
|
|
