Molecular weight (DA)
90kD
Immunogen
The antiserum was produced against synthesized peptide derived from human RNF19A. AA range:51-100
Specificity
Dorfin Polyclonal Antibody detects endogenous levels of Dorfin protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes a member of the ring between ring fingers (RBR) protein family, and the encoded protein contains two RING-finger motifs and an in between RING fingers motif. This protein is an E3 ubiquitin ligase that is localized to Lewy bodies, and ubiquitylates synphilin-1, which is an interacting protein of alpha synuclein in neurons. The encoded protein may be involved in amyotrophic lateral sclerosis and Parkinson's disease. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013],
Function
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.,pathway:Protein modification; protein ubiquitination.,PTM:Phosphorylated upon DNA damage, probably by ATM or ATR.,similarity:Belongs to the RBR family. RNF19 subfamily.,similarity:Contains 1 IBR-type zinc finger.,similarity:Contains 2 RING-type zinc fingers.,subcellular location: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.,subunit:Interacts with UBE2L3 and UBE2L6. Interac
Protein name
E3 ubiquitin-protein ligase RNF19A
Other name
RNF19A; RNF19; E3 ubiquitin-protein ligase RNF19A; Double ring-finger protein; Dorfin; RING finger protein 19A; p38
Human protein sequence Database
Q9NV58
Mouse protein sequence database
P50636
Rat protein sequence database
Cellular localization
Membrane ; Multi-pass membrane protein . Cytoplasm, cytoskeleton, microtubule organizing center, centrosome . 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 expression
Widely expressed, with highest levels in heart. Ubiquitously expressed in the central nervous system.
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