E3 ubiquitin-protein ligase RNF213 (UniProt: Q63HN8; also known as ALK lymphoma oligomerization partner on chromosome 17, Mysterin, RING finger protein 213, RING-type E3 ubiquitin transferase RNF21) is encoded by the RNF213 (also known as ALO17, C17orf27, KIAA1554, KIAA1618, MYSTR) gene (Gene ID: 57674) in human. RNF213 is a widely expressed, homooligomeric, cytoplasmic protein that has E3 ubiquitin-protein ligase and AAA+ ATPase activity. It contains a zinc-finger domain (aa 3997-4036) that is required for its ubiquitin-protein ligase activity. RNF213 is shown to be involved in angiogenesis and in non-canonical Wnt signaling pathway in vascular development. Four isoforms of RNF213 have been described that are produced by alternative splicing. RNF213 ligase activity is negatively regulated by PTP1B in HER2+ breast cancer cells and RNF213 knockdown is shown to reverse the effects of PTP1B deficiency on alpha-keto-dependent dehydrogenases, non-mitochondrial oxygen consumption, and hypoxia-induced death of HER2+ BC cells. RNF213 activity is down-regulated by let-7c miRNA, which binds to the 3'-UTR transcript of RNF213. Microbial infection leading to induction of pro-inflammatory cytokines are shown to up-regulate its activity. Defects in RNF213 gene are known to cause Moyamoya disease 2, a progressive cerebral angiopathy characterized by bilateral intracranial carotid artery stenosis and telangiectatic vessels in the region of the basal ganglia, which can lead to transient ischemia and/or rupture of the collateral vessel. (Ref.: Banh, RS et al (2016). Nat. Cell Biol. 18(7); 803-813).
Synonyms: E3 ubiquitin-protein ligase RNF213, ALK lymphoma oligomerization partner on chromosome 17, Mysterin, RING finger protein 213, RING-type E3 ubiquitin transferase RNF213
Application: Immunoprecipitation Analysis: A representative lot detected Ring Finger Protein 213 (RNF213) in BT474, MDA-MB-361, and HCC1954 cells (Banh, R.S., et. al. (2016). Nat Cell Biol. 18(7):803-13).Western Blotting Analysis: A 1:2,000 dilution from a representative lot detected Ring Finger Protein 213 (RNF213) in HUVEC transfected with siRNA213 (Courtesy of Dr Akio Koizumi at Kyoto University).Immunocytochemistry Analysis: A representative lot detected Ring Finger Protein 213 (RNF213) in HeLa cells treated with RNF213 siRNA (Hitomi, T., et. al. (2013). Biochem Biophys Res Commun. 438(1):13-9).Western Blotting Analysis: A representative lot detected Ring Finger Protein 213 (RNF213) in Western Blotting applications (Hitomi, T., et. al. (2013). Biochem Biophys Res Commun. 438(1):13-9; Banh, R.S., et. al. (2016). Nat Cell Biol. 18(7):803-13; Kobayashi, H., et. al. (2015). J Am Heart Assoc. 4(7)).
Other Notes: Concentration: Please refer to lot specific datasheet.