catalog number :
MBS241650
products full name :
Goat Polyclonal to Human RNF213
products short name :
RNF213
products name syn :
Anti-RNF213 Antibody (Internal) IHC-plus; RNF213; C17orf27; KIAA1618; KIAA1554; Mysterin; MYSTR; ALO17; Moyamoya disease 2; MYMY2; NET57; Ring finger protein 213; Human RNF213
other names :
E3 ubiquitin-protein ligase RNF213 isoform 3; E3 ubiquitin-protein ligase RNF213; E3 ubiquitin-protein ligase RNF213; mysterin; ALK lymphoma oligomerization partner on chromosome 17; ring finger protein 213; ALK lymphoma oligomerization partner on chromosome 17; Mysterin; RING finger protein 213
products gene name :
RNF213
other gene names :
RNF213; RNF213; ALO17; MYMY2; MYSTR; NET57; C17orf27; KIAA1618; ALO17; C17orf27; KIAA1554; KIAA1618; MYSTR
uniprot entry name :
RN213_HUMAN
reactivity :
Human. Predicted Reactivity: Gorilla (at least 90% immunogen sequence identity)
specificity :
Human RNF213.
purity :
Immunoaffinity Purified
form :
Tris-buffered saline, pH 7.3, 0.5% BSA, 0.02% sodium azide
concentration :
0.5 mg/ml
storage stability :
Store at -20 degree C. Minimize freezing and thawing.
tested application :
Immunohistochemistry (IHC - Paraffin), ELISA (EIA)
app notes :
ELISA (1:128000), IHC-P (1.25 ug/ml). Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary anti.
other info1 :
Target Species: Human. Immunogen Description: Synthetic peptide C-KNPQTQTEERFRPQ from an internal region of human RNF213 (NP_065965.3). Percent identity with other species by BLAST analysis: Human (100%), Gorilla (93%).
other info2 :
Immunogen Type: Synthetic peptide. Immunogen: RNF213 antibody was raised against synthetic peptide C-KNPQTQTEERFRPQ from an internal region of human RNF213 (NP_065965.3). Percent identity with other species by BLAST analysis: Human (100%), Gorilla (93%). Antigen Modification: Internal
ncbi acc num :
NP_001243000.2
ncbi gb acc num :
NM_001256071.2
ncbi mol weight :
64,260 Da
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: Ubiquitin conjugating system; EC 6.3.2.-; Oncoprotein. Chromosomal Location of Human Ortholog: 17q25.3. Cellular Component: membrane; cytoplasm; nucleolus. Molecular Function: zinc ion binding; ATPase activity; ubiquitin-protein ligase activity; ligase activity. Biological Process: protein autoubiquitination; protein ubiquitination