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phospho-NBN (Ser343) Rabbit pAb (bs-6125R)  
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產品編號 bs-6125R
英文名稱 phospho-NBN (Ser343) Rabbit pAb
中文名稱 磷酸化DNA修復蛋白NBS1抗體
別    名 p95 NBS1(phospho S343); p95 NBS1(phospho Ser343); p-p95 NBS1(phospho S343); p95 NBS1; Nijmegen breakage syndrome 1; Nijmegen breakage syndrome 1(nibrin); AT V1; AT V2; AT-V1; AT-V2; ATV; Cell cycle regulatory protein p95; NBS 1; NBS; NBS1; Nibrin; Nijmegen breakage syndrome; Nijmegen breakage syndrome protein 1; p95; NBN_HUMAN; Nijmegen breakage syndrome protein 1.  
產品類型 磷酸化抗體 
研究領域 腫瘤  細胞生物  染色質和核信號  細胞周期蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human (predicted: Rat,Rabbit)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 85 kDa
檢測分子量
細胞定位 細胞核 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthesised phosphopeptide derived from human NBS1 around the phosphorylation site of Ser343: SL(p-S)QG 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 Component of the MRE11/RAD50/NBN (MRN complex) which plays a critical role in the cellular response to DNA damage and the maintenance of chromosome integrity. The complex is involved in double-strand break (DSB) repair, DNA recombination, maintenance of telomere integrity, cell cycle checkpoint control and meiosis. The complex possesses single-strand endonuclease activity and double-strand-specific 3'-5' exonuclease activity, which are provided by MRE11A. RAD50 may be required to bind DNA ends and hold them in close proximity. NBN modulate the DNA damage signal sensing by recruiting PI3/PI4-kinase family members ATM, ATR, and probably DNA-PKcs to the DNA damage sites and activating their functions. It can also recruit MRE11 and RAD50 to the proximity of DSBs by an interaction with the histone H2AX. NBN also functions in telomere length maintenance by generating the 3' overhang which serves as a primer for telomerase dependent telomere elongation. NBN is a major player in the control of intra-S-phase checkpoint and there is some evidence that NBN is involved in G1 and G2 checkpoints. The roles of NBS1/MRN encompass DNA damage sensor, signal transducer, and effector, which enable cells to maintain DNA integrity and genomic stability.

Function:
Component of the MRE11-RAD50-NBN (MRN complex) which plays a critical role in the cellular response to DNA damage and the maintenance of chromosome integrity. The complex is involved in double-strand break (DSB) repair, DNA recombination, maintenance of telomere integrity, cell cycle checkpoint control and meiosis. The complex possesses single-strand endonuclease activity and double-strand-specific 3'-5' exonuclease activity, which are provided by MRE11A. RAD50 may be required to bind DNA ends and hold them in close proximity. NBN modulate the DNA damage signal sensing by recruiting PI3/PI4-kinase family members ATM, ATR, and probably DNA-PKcs to the DNA damage sites and activating their functions. It can also recruit MRE11 and RAD50 to the proximity of DSBs by an interaction with the histone H2AX. NBN also functions in telomere length maintenance by generating the 3' overhang which serves as a primer for telomerase dependent telomere elongation. NBN is a major player in the control of intra-S-phase checkpoint and there is some evidence that NBN is involved in G1 and G2 checkpoints. The roles of NBS1/MRN encompass DNA damage sensor, signal transducer, and effector, which enable cells to maintain DNA integrity and genomic stability. Forms a complex with RBBP8 to link DNA double-strand break sensing to resection.

Subunit:
Component of the MRN complex composed of two heterodimers RAD50/MRE11A associated with a single NBN. Component of the BASC complex, at least composed of BRCA1, MSH2, MSH6, MLH1, ATM, BLM, RAD50 and MRE11A (By similarity). Interacts with histone H2AFX this requires phosphorylation of H2AFX on 'Ser-139'. Interacts with HJURP, INTS3, KPNA2 and TERF2. Interacts with RBBP8; the interaction links the role of the MRN complex in DNA double-strand break sensing to resection. Interacts with SP100; recruits NBN to PML bodies.

Subcellular Location:
Nucleus. Nucleus, PML body. Chromosome, telomere. Note=Localizes to discrete nuclear foci after treatment with genotoxic agents.

Tissue Specificity:
Ubiquitous. Expressed at high levels in testis.

Post-translational modifications:
Phosphorylated by ATM in response of ionizing radiation, and such phosphorylation is responsible intra-S phase checkpoint control and telomere maintenance.

DISEASE:
Nijmegen breakage syndrome (NBS) [MIM:251260]: A disorder characterized by chromosomal instability, radiation sensitivity, microcephaly, growth retardation, immunodeficiency and predisposition to cancer, particularly to lymphoid malignancies. Note=The disease is caused by mutations affecting the gene represented in this entry.
Breast cancer (BC) [MIM:114480]: A common malignancy originating from breast epithelial tissue. Breast neoplasms can be distinguished by their histologic pattern. Invasive ductal carcinoma is by far the most common type. Breast cancer is etiologically and genetically heterogeneous. Important genetic factors have been indicated by familial occurrence and bilateral involvement. Mutations at more than one locus can be involved in different families or even in the same case. Note=Disease susceptibility is associated with variations affecting the gene represented in this entry.
Aplastic anemia (AA) [MIM:609135]: A form of anemia in which the bone marrow fails to produce adequate numbers of peripheral blood elements. It is characterized by peripheral pancytopenia and marrow hypoplasia. Note=Disease susceptibility may be associated with variations affecting the gene represented in this entry.
Note=Defects in NBN might play a role in the pathogenesis of childhood acute lymphoblastic leukemia (ALL).

Similarity:
Contains 1 BRCT domain.
Contains 1 FHA domain.

SWISS:
O60934

Gene ID:
4683

Database links:

Entrez Gene: 4683 Human

Entrez Gene: 27354 Mouse

Entrez Gene: 85482 Rat

Omim: 602667 Human

SwissProt: O60934 Human

SwissProt: Q9R207 Mouse

SwissProt: Q9JIL9 Rat

Unigene: 492208 Human

Unigene: 20866 Mouse

Unigene: 25214 Rat



產品圖片
Sample: Lane 1: Human K562 cell lysates Lane 2: Human HepG2 cell lysates Lane 3: Human 293T cell lysates Lane 4: Human A549 cell lysates Primary: Anti-phospho-NBN (Ser343) (bs-6125R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 85 kDa Observed band size: 100 kDa
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