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Rabbit Anti-DNA Ligase IV  antibody (bsm-54131R)  
~~~促銷代碼KT202411~~~
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產品編號 bsm-54131R
英文名稱 Rabbit Anti-DNA Ligase IV  antibody
中文名稱 DNA連接酶4重組兔單抗
別    名 LIG4; DNA ligase IV ATP dependent; DNA joinase; DNA joinase; DNA ligase 4; DNA ligase IV; DNA repair enzyme; DNA repair enzyme; DNLI4_HUMAN; LIG 4; LIG4; LIG-4; Ligase IV; Ligase IV; Ligase IV DNA ATP dependent; Polydeoxyribonucleotide synthase [ATP] 4; Polydeoxyribonucleotide synthase 4; Polydeoxyribonucleotide synthase; Polydeoxyribonucleotide synthase; Polynucleotide ligase; Polynucleotide ligase; Sealase.  
研究領域 細胞生物  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Recombinant
克 隆 號 2D9
交叉反應 Human
產品應用 WB=1:500-2000,IHC-P=1:50-200,IHC-F=1:400-800,ICC/IF=1:10-50,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 104kDa
細胞定位 細胞核 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 Recombinant human DNA Ligase IV 
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 The X-ray repair cross-complementing protein XRCC4 and DNA Ligase IV are essential for repairing double-strand breaks in DNA. These proteins form a critical complex consisting of two molecules of each protein that preferentially bind DNA with nicks or broken ends. As an obligate accessory molecule, XRCC4 binds to DNA Ligase IV and enhances its joining activity. The XRCC4/ DNA Ligase IV complex is also involved in V(D)J recombination. V(D)J recombination occurs in normal development of the adaptive immune system and involves the formation of a double-strand break intermediate. Deletions of either DNA Ligase IV or XRCC4 inhibit the completion of V(D)J recombination, resulting in a high incidence of apoptosis in the developing nervous system and a block in B and T cell maturation.

Function:
Efficiently joins single-strand breaks in a double-stranded polydeoxynucleotide in an ATP-dependent reaction. Involved in DNA non-homologous end joining (NHEJ) required for double-strand break repair and V(D)J recombination. The LIG4-XRCC4 complex is responsible for the NHEJ ligation step, and XRCC4 enhances the joining activity of LIG4. Binding of the LIG4-XRCC4 complex to DNA ends is dependent on the assembly of the DNA-dependent protein kinase complex DNA-PK to these DNA ends.

Subcellular Location:
Testis, thymus, prostate and heart.

DISEASE:
Defects in LIG4 are the cause of LIG4 syndrome (LIG4S) [MIM:606593]. This disease is characterized by immunodeficiency and developmental and growth delay. Patients display unusual facial features, microcephaly, growth and/or developmental delay, pancytopenia, and various skin abnormalities.
Defects in LIG4 are a cause of severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-positive with sensitivity to ionizing radiation (RSSCID) [MIM:602450]. SCID refers to a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients with SCID present in infancy with recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development. Individuals affected by RS-SCID show defects in the DNA repair machinery necessary for coding joint formation and the completion of V(D)J recombination. A subset of cells from such patients show increased radiosensitivity.

Similarity:
Belongs to the ATP-dependent DNA ligase family.
Contains 2 BRCT domains.

SWISS:
P49917

Gene ID:
3981

Database links:

Entrez Gene: 3981 Human

Entrez Gene: 319583 Mouse

Entrez Gene: 100173511 Orangutan

Omim: 601837 Human

SwissProt: P49917 Human

SwissProt: Q8BTF7 Mouse

SwissProt: Q5R6L3 Orangutan

Unigene: 166091 Human

Unigene: 80584 Mouse



產品圖片
Sample: Lane 1: Human MCF-7 cell lysates Lane 2: Human Jurkat cell lysates Lane 3: Human HT-1080 cell lysates Lane 4: Human HeLa cell lysates Lane 5: Human Ramos cell lysates Lane 6: Human HepG2 cell lysates Primary: Anti-DNA Ligase IV (bsm-54131R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 104 kDa Observed band size: 104 kDa
Paraformaldehyde-fixed, paraffin embedded (human placenta tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (DNA Ligase IV) Monoclonal Antibody, Unconjugated (bsm-54131R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (human colon cancer); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (DNA Ligase IV) Monoclonal Antibody, Unconjugated (bsm-54131R) at 1:50 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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