環(huán)指蛋白213說明書 英文名稱  Anti-RNF213

中文名稱  環(huán)指蛋白213說明書 

別    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.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Pig, Cow, Horse, Sheep

產(chǎn)品類型   一抗  

研究領(lǐng)域     細(xì)胞生物 免疫學(xué)

蛋白分子量  predicted molecular weight: 591kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human RNF213

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

環(huán)指蛋白213說明書 產(chǎn)品應(yīng)用   ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200

（石蠟切片需做抗原修復(fù)） 

 not yet tested in other applications.

 optimal dilutions/concentrations should be determined by the end user.  

保存條件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

產(chǎn)品介紹 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 ubiquitin-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 hemorrhage. Hemiplegia of sudden onset and epileptic seizures constitute the prevailing presentation in childhood, while subarachnoid bleeding occurs more frequently in adults.

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 : UniProtKB/Swiss-Prot: Q63HN8.3

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