|Other Names||E3 ubiquitin-protein ligase RNF19A, 632-, Double ring-finger protein, Dorfin, RING finger protein 19A, p38, RNF19A, RNF19|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||E3 ubiquitin-protein ligase which accepts ubiquitin from E2 ubiquitin-conjugating enzymes UBE2L3 and UBE2L6 in the form of a thioester and then directly transfers the ubiquitin to targeted substrates, such as SNCAIP or CASR. Specifically ubiquitinates pathogenic SOD1 variants, which leads to their proteasomal degradation and to neuronal protection.|
|Cellular Location||Membrane; Multi-pass membrane protein. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Note=Present in the hyaline inclusion bodies specifically found in motor neurons from amyotrophic lateral sclerosis patients. Present in the Lewy bodies specifically found in neurons from Parkinson disease patients|
|Tissue Location||Widely expressed, with highest levels in heart. Ubiquitously expressed in the central nervous system|
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The protein encoded this gene contains two RING-fingermotifs and an IBR (in between RING fingers) motif. This protein isan E3 ubiquintin ligase that is localized in Lewy bodies (LBs), acharacteristic neuronal inclusion in Parkinson's disease (PD)brains. This protein interacts with UBE2L3/UBCH7 and UBE2E2/UBCH8,but not other ubiquitin-conjugating enzymes. This protein is foundto bind and ubiquitylate synphilin 1 (SNCAIP), which is ainteracting protein of alpha synuclein in neurons, and a majorcomponent of LB. Alternatively spliced transcript variants encodingthe same protein have been reported.
Huh, J.W., et al. Gene 424 (1-2), 63-70 (2008) :Matsuoka, S., et al. Science 316(5828):1160-1166(2007)Ishigaki, S., et al. Neurobiol. Dis. 25(2):331-341(2007)Huang, Y., et al. J. Biol. Chem. 281(17):11610-11617(2006)Ishigaki, S., et al. J. Biol. Chem. 279(49):51376-51385(2004)
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