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核苷酸還原酶M2B抗體品牌 英文名稱  Anti-p53R2/RRM2B

中文名稱  核苷酸還原酶M2B抗體品牌 

別    名 DKFZp686M05248; MGC102856; MGC42116; MTDPS8A; MTDPS8B; p53 inducible ribonucleotide reductase small subunit 2 homolog; p53 inducible ribonucleotide reductase small subunit 2 like protein; p53 R2; p53-inducible ribonucleotide reductase small subunit 2-like protein; p53R2; Ribonucleoside diphosphate reductase M2 subunit B; Ribonucleoside-diphosphate reductase subunit M2 B; Ribonucleotide reductase M2 B (TP53 inducible); Ribonucleotide reductase M2 B; Ribonucleotide reductase small subunit like 2 p53 inducible; RIR2B_HUMAN; RRM 2B; RRM2B; TP53 inducible ribonucleotide reductase M2 B; TP53-inducible ribonucleotide reductase M2 B.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.1ml/100μg 0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

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

產(chǎn)品類型   一抗  

研究領(lǐng)域     免疫學(xué) 染色質(zhì)和核信號(hào) 信號(hào)轉(zhuǎn)導(dǎo) 表觀遺傳學(xué)

蛋白分子量  predicted molecular weight: 41kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human p53R2/RRM2B

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide

核苷酸還原酶M2B抗體品牌 產(chǎn)品應(yīng)用   WB=1:100-500 ELISA=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500

（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 This gene encodes the small subunit of a p53-inducible ribonucleotide reductase. This heterotetrameric enzyme catalyzes the conversion of ribonucleoside diphosphates to deoxyribonucleoside diphosphates. The product of this reaction is necessary for DNA synthesis. Mutations in this gene have been associated with autosomal recessive mitochondrial DNA depletion syndrome, autosomal dominant progressive external ophthalmoplegia-5, and mitochondrial neurogastrointestinal encephalopathy. Alternatively spliced transcript variants have been described.[provided by RefSeq, Feb 2010].

Function : Plays a pivotal role in cell survival by repairing damaged DNA in a p53/TP53-dependent manner. Supplies deoxyribonucleotides for DNA repair in cells arrested at G1 or G2. Contains an iron-tyrosyl free radical center required for catalysis. Forms an active ribonucleotide reductase (RNR) complex with RRM1 which is expressed both in resting and proliferating cells in response to DNA damage.

Subunit : Heterotetramer with large (RRM1) subunit. Interacts with p53/TP53. Interacts with RRM1 in response to DNA damage.

Subcellular Location : Cytoplasm. Nucleus. Translocates from cytoplasm to nucleus in response to DNA damage.

DISEASE : Mitochondrial DNA depletion syndrome 8A (MTDPS8A) [MIM:612075]: A disorder due to mitochondrial dysfunction characterized by various combinations of neonatal hypotonia, neurological deterioration, respiratory distress, lactic acidosis, and renal tubulopathy. Note=The disease is caused by mutations affecting the gene represented in this entry.

Mitochondrial DNA depletion syndrome 8B (MTDPS8B) [MIM:612075]: A disease due to mitochondrial dysfunction and characterized by ophthalmoplegia, ptosis, gastrointestinal dysmotility, cachexia, peripheral neuropathy. Note=The disease is caused by mutations affecting the gene represented in this entry.

Progressive external ophthalmoplegia with mitochondrial DNA deletions autosomal dominant 5 (PEOA5) [MIM:613077]: A disorder characterized by progressive weakness of ocular muscles and levator muscle of the upper eyelid. In a minority of cases, it is associated with skeletal myopathy, which predominantly involves axial or proximal muscles and which causes abnormal fatigability and even permanent muscle weakness. Ragged-red fibers and atrophy are found on muscle biopsy. A large proportion of chronic ophthalmoplegias are associated with other symptoms, leading to a multisystemic pattern of this disease. Additional symptoms are variable, and may include cataracts, hearing loss, sensory axonal neuropathy, ataxia, depression, hypogonadism, and parkinsonism. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity : Belongs to the ribonucleoside diphosphate reductase small chain family.

Database links : UniProtKB/Swiss-Prot: Q7LG56.1

Involvement in disease:Defects in RRM2B are the cause of mitochondrial DNA depletion syndrome type 8A (MTDPS8A). A disorder due to mitochondrial dysfunction characterized by various combinations of neonatal hypotonia, neurological deterioration, respiratory distress, lactic acidosis, and renal tubulopathy.

Defects in RRM2B are the cause of mitochondrial DNA depletion syndrome type 8B (MTDPS8B). A disease due to mitochondrial dysfunction and characterized by ophthalmoplegia, ptosis, gastrointestinal dysmotility, cachexia, peripheral neuropathy.

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