环指蛋白213
规格：1尘驳/1尘濒
英文名： RNF213
别名： C17orf27; ALK lymphoma oligomerization partner on chromosome 17; ALO17_HUMAN; Chromosome 17 open reading frame 27; NET57; Protein ALO17; Q63HN8; RING finger protein 213; RNF 213; RNF213.
分子量： 591kD
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human RNF213
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse, Sheep,
细胞定位：细胞浆
环指蛋白213产物介绍：background: The RING-type zinc finger motif is present in a number of viral and eukaryotic proteins and is made up of a conserved cysteine-rich domain that is able to bind two zinc atoms. Proteins that contain this conserved domain are generally involved in the ubiquitination pathway of protein degradation. RNF213 (ring finger protein 213), also known as NET57, is a 3,280 amino acid protein containing one RING-type zinc finger domain through which it may play a role in transcriptional regulation and protein degradation. RNF213 is encoded by a gene located on human chromosome 17, which comprises over 2.5% of the human genome and encodes over 1,200 genes. Two key tumor suppressor genes are associated with chromosome 17, namely, p53 and BRCA1. Tumor suppressor p53 is necessary for maintenance of cellular genetic integrity by moderating cell fate through DNA repair versus cell death. Malfunction or loss of p53 expression is associated with malignant cell growth and Li-Fraumeni syndrome. Like p53, BRCA1 is directly involved in DNA repair, though specifically it is recognized as a genetic determinant of early onset breast cancer and predisposition to cancers of the ovary, colon, prostate gland and fallopian tubes. Function: Probable E3环指蛋白213ubiquitin-protein ligase that may play a role in angiogenesis. May also have an ATPase activity. Subcellular Location: Cytoplasm. Tissue Specificity: Widely expressed (at protein level). Post-translational modifications: Autoubiquitinates. DISEASE: Defects in RNF213 are the cause of susceptibility to Moyamoya disease type 2 (MYMY2) [MIM:607151]. 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 环指蛋白213hemorrhage. Hemiplegia of sudden onset and epileptic seizures constitute the prevailing presentation in childhood, while subarachnoid bleeding occurs more frequently in s. Note=A chromosomal aberration involving ALO17 is associated with anaplastic large-cell lymphoma (ALCL). Translocation t(2;17)(p23;q25) with ALK. Similarity: Contains 1 RING-type zinc finger. Database links: Entrez Gene: 57674 Human Omim: 613768 Human SwissProt: Q9HCF4 Human Unigene: 195642 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
环指蛋白213产物应用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid