英文名称    DNA Ligase IV    

中文名称    DNA连接酶4抗体    

别    名    LIG4; DNA ligase IV ATP dependent; 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.    

说 明 书    0.1ml  0.2ml      

研究领域    细胞生物  表观遗传学      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep,     

产品应用    WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 DNA连接酶4抗体（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.    

分 子 量    104kDa    

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human DNA Ligase IV    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存条件    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.    

DNA连接酶4抗体产品介绍    background:

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.

Tissue Specificity:
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.    

DNA连接酶4抗体

hz-0297M-RBIThz Mouse Anti-human IgG/RBITC  罗丹明标记的小鼠抗人IgG

hz-0297M-Gold Mouse Anti-human IgG/Gold  胶体金标记的小鼠抗人IgG

hz-0345M-FIThz Mouse Anti-human IgM/FITC  FITC标记的小鼠抗人IgM

hz-0345M-hzy3 Mouse Anti-human IgM/Cy3  Cy3标记的小鼠抗人IgM

hz-0345M-hzy5 Mouse Anti-human IgM/Cy5  Cy5标记的小鼠抗人IgM

hz-0345M-hzy5.5 Mouse Anti-human IgM/Cy5.5  Cy5.5标记的小鼠抗人IgM

hz-0345M-PE-hzy3 Mouse Anti-human IgM/PE-Cy3  PE-Cy3标记的小鼠抗人IgM

hz-0345M-PE-hzY5 Mouse Anti-human IgM/PE-CY5  PE-CY5标记的小鼠抗人IgM

hz-0345M-APhz Mouse Anti-human IgM/APC  APC标记的小鼠抗人IgM

hz-0345M-AF350 Mouse Anti-human IgM/Alexa Fluor 350  Alexa Fluor 350标记的小鼠抗人IgM

hz-0345M-AF488 Mouse Anti-human IgM/Alexa Fluor 488  Alexa Fluor 488标记的小鼠抗人IgM

hz-0345M-AF555 Mouse Anti-human IgM/Alexa Fluor 555  Alexa Fluor 555标记的小鼠抗人IgM

hz-0345M-AF647 Mouse Anti-human IgM/Alexa Fluor 647  Alexa Fluor 647标记的小鼠抗人IgM

hz-0345M-PE-hzy5.5 Mouse Anti-human IgM/PE-Cy5.5  PE-Cy5.5标记的小鼠抗人IgM

hz-0345M-PE-hzy7 Mouse Anti-human IgM/PE-Cy7  PE-Cy7标记的小鼠抗人IgM

hz-0345M-RBIThz Mouse Anti-human IgM/RBITC  罗丹明标记的小鼠抗人IgM

hz-0345M-Bio Mouse Anti-human IgM/Bio  生物素标记的小鼠抗人IgM

hz-0345M-HRP Mouse Anti-human IgM/HRP  辣根过氧化物酶标记的小鼠抗人IgM

hz-0345M-Gold Mouse Anti-human IgM/Gold  胶体金标记的小鼠抗人IgM

hz-0345M-AP Mouse Anti-human IgM/AP  碱性磷酸酶（AP）标记的小鼠抗人IgM

hz-0345M-hzy7 Mouse Anti-human IgM/Cy7  Cy7标记的小鼠抗人IgM

hz-0345M-PE Mouse Anti-human IgM/PE  PE标记的小鼠抗人IgM

hz-0294M-HRP Mouse Anti-Goat IgG/HRP  辣根过氧化物酶标记的小鼠抗羊IgG

hz-0294M-RBIThz Mouse Anti-Goat IgG/RBITC  罗丹明标记的小鼠抗羊IgG

hz-0294M-Bio Mouse Anti-Goat IgG/Bio  生物素标记的小鼠抗羊IgG

hz-0294M-Gold Mouse Anti-Goat IgG/Gold  胶体金标记的小鼠抗羊IgG

hz-0294M-hzy3 Mouse Anti-Goat IgG/Cy3  Cy3标记的小鼠抗羊IgG

hz-0294M-hzy5 Mouse Anti-Goat IgG/Cy5  Cy5标记的小鼠抗羊IgG

hz-0294M-hzy5.5 Mouse Anti-Goat IgG/Cy5.5  Cy5.5标记的小鼠抗羊IgG

hz-0294M-hzy7 Mouse Anti-Goat IgG/Cy7  Cy7标记的小鼠抗羊IgG

hz-0294M-PE Mouse Anti-Goat IgG/PE  PE标记的小鼠抗羊IgG

hz-0294M-PE-hzy3 Mouse Anti-Goat IgG/PE-Cy3  PE-Cy3标记的小鼠抗羊IgG

hz-0294M-PE-hzY5 Mouse Anti-Goat IgG/PE-CY5  PE-CY5标记的小鼠抗羊IgG