The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 0.5 µg/ml. Detects a band of approximately 48 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.
Acts as an E2-dependent E3 ubiquitin-protein ligase, involved in innate immune defense against viruses. Ubiquitinates DDX58 and is required for full activation of the DDX58 signaling resulting in interferon beta production.
Expressed in skeletal muscle, spleen, kidney, placenta, prostate, stomach, thyroid and tongue. Also weakly expressed in heart, thymus, liver and lung.
Protein modification; protein ubiquitination.
Involvement in disease
Defects in RNF135 are the cause of macrocephaly macrosomia facial dysmorphism syndrome (MMFD) [MIM:614192]. MMFD is an autosomal dominant disorder characterized by the association of macrothrombocytopathy and progressive sensorineural hearing loss without renal dysfunction.
Hu J et al. A common polymorphism in the caspase recruitment domain of RIG-I modifies the innate immune response of human dendritic cells. J Immunol185:424-32 (2010).
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