catalog number :
MBS420069
products full name :
Goat anti-RNF213/C17orf27 (C Terminus) Antibody
products short name :
RNF213/C17orf27
products name syn :
Goat Anti-RNF213 / C17orf27 (internal) Antibody; RNF213; C17orf27; ring finger protein 213; chromosome 17 open reading frame 27; KIAA1554; RNF213 antibody; C17orf27 antibody; ring finger protein 213 antibody; chromosome 17 open reading frame 27 antibody; KIAA1554 antibody; RNF213 / C17orf27 (C Terminus)
other names :
E3 ubiquitin-protein ligase RNF213; E3 ubiquitin-protein ligase RNF213; ring finger protein 213; ALK lymphoma oligomerization partner on chromosome 17
products gene name :
RNF213
products gene name syn :
C17orf27
other gene names :
RNF213; RNF213; ALO17; MYMY2; MYSTR; NET57; C17orf27; KIAA1618
uniprot entry name :
RN213_HUMAN
reactivity :
Tested: Human; Expected from sequence similarity: Human
purity :
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide
form :
Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
concentration :
100ug specific antibody in 200ul
storage stability :
Aliquot and store at -20 degree C. Minimize freezing and thawing.
tested application :
Peptide ELISA (EIA), Immunohistochemistry (IHC)
app notes :
Peptide ELISA: Antibody detection limit dilution 1: 4000. Immunohistochemistry: In paraffin embedded human kidney shows rare staining of nucleoli across the kidney. Recommended concentration, 2.5ug/ml. Western Blot: Not yet tested - our routinely used western blotting protocol does not allow detection of proteins as large as the calculated size of 374kDa according to NP_065965.3. Therefore we cannot recommend an optimal concentration and the product is investigative grade.
other info1 :
Immunogen: Peptide with sequence C-KTAAVLKWNRE, from the C Terminus of the protein sequence according to NP_065965.4. Epitope: C Terminus
ncbi acc num :
NP_065965.4
ncbi gb acc num :
NM_020914.4
ncbi mol weight :
64,260 Da
ncbi pathways :
Adaptive Immune System Pathway (1269171); Antigen Processing: Ubiquitination Proteasome Degradation Pathway (1269193); Class I MHC Mediated Antigen Processing Presentation Pathway (1269192); Immune System Pathway (1269170)
ncbi summary :
This gene encodes a protein containing a C3HC4-type RING finger domain, which is a specialized type of Zn-finger that binds two atoms of zinc and is thought to be involved in mediating protein-protein interactions. The protein also contains an AAA domain, which is associated with ATPase activity. This gene is a susceptibility gene for Moyamoya disease, a vascular disorder of intracranial arteries. This gene is also a translocation partner in anaplastic large cell lymphoma and inflammatory myofibroblastic tumor cases, where a t(2;17)(p23;q25) translocation has been identified with the anaplastic lymphoma kinase (ALK) gene on chromosome 2, and a t(8;17)(q24;q25) translocation has been identified with the MYC gene on chromosome 8. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2011]
uniprot summary :
RNF213: Probable E3 ubiquitin-protein ligase that may play a role in angiogenesis. May also have an ATPase activity. Defects in RNF213 are the cause of susceptibility to Moyamoya disease type 2 (MYMY2). A progressive cerebral angiopathy characterized by bilateral intracranial carotid artery stenosis and telangiectatic vessels in the region of the basal ganglia. The abnormal vessels resemble a 'puff of smoke' (moyamoya) on cerebral angiogram. Affected individuals can develop transient ischemic attacks and/or cerebral infarction, and rupture of the collateral vessels can cause intracranial hemorrhage. Hemiplegia of sudden onset and epileptic seizures constitute the prevailing presentation in childhood, while subarachnoid bleeding occurs more frequently in adults. A chromosomal aberration involving ALO17 is associated with anaplastic large-cell lymphoma (ALCL). Translocation t(2;17)(p23;q25) with ALK. 4 isoforms of the human protein are produced by alternative splicing. Protein type: EC 6.3.2.-; Oncoprotein; Ubiquitin conjugating system. Chromosomal Location of Human Ortholog: 17q25.3. Cellular Component: cytoplasm; cytosol; membrane; nucleolus. Molecular Function: ATPase activity; ligase activity; protein binding; ubiquitin-protein ligase activity; zinc ion binding. Biological Process: angiogenesis; protein autoubiquitination; protein homooligomerization; protein polyubiquitination; protein ubiquitination; sprouting angiogenesis; ubiquitin-dependent protein catabolic process