DNA Ligase IV antibody

¥1380 - 2200
康朗生物
中国/美国/德国
2022-03-24 21:09

上海康朗生物科技有限公司

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上海康朗生物科技有限公司
蔡盼
18217752821 021-61998208
shklbio@163.com
产品属性
供应商上海康朗生物科技有限公司
数量大量
目录编号kl-1677R
克隆性多克隆
抗原来源Rabbit
保质期12个月
抗体英文名DNA Ligase IV antibody
抗体名DNA连接酶4抗体
宿主Rabbit
适应物种Human, Mouse, Rat, Chicken, Dog, Cow, Horse, Rabbit, Sheep,
免疫原KLH conjugated synthetic peptide derived from human DNA Ligase IV:51-150/911
亚型IgG
形态冻干粉或液体
应用范围WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500
浓度1mg/ml
保存条件-20 °C
规格100ul 200ul
产品说明
DNA Ligase IV antibody
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.
规格价格100ul/1380元 购买 200ul/2200元 购买 大包装/询价
说 明 书100ul 200ul
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=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (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/ml
免 疫 原KLH conjugated synthetic peptide derived from human DNA Ligase IV:51-150/911
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
PubMedPubMed
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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 
产品图片
Tissue/cell: human laryngocarcinoma; 4% Paraformaldehyde-fixed and paraffin-embedded; 
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; 
Incubation: Anti-LIG4 Polyclonal Antibody, Unconjugated(bs-1677R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining