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Rabbit Anti-LIG4 Recombinant Antibody (EG981) (CBMAB-EN1163-LY)

The product is antibody recognizes DNL4. The antibody EG981 immunoassay techniques such as: WB: 1:500~1:1000 ELISA: 1:20000.
See all LIG4 antibodies

Summary

Host Animal
Rabbit
Specificity
Human
Clone
EG981
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 ELISA: 1:20000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human DNL4.
Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
ligase IV, DNA, ATP-dependent
Entrez Gene ID
3981
UniProt ID
P49917
Function
DNA ligase involved in DNA non-homologous end joining (NHEJ); required for double-strand break (DSB) repair and V(D)J recombination (PubMed:8798671, PubMed:9242410, PubMed:9809069, PubMed:12517771, PubMed:17290226).
Catalyzes the NHEJ ligation step of the broken DNA during DSB repair by resealing the DNA breaks after the gap filling is completed (PubMed:9242410, PubMed:9809069, PubMed:12517771, PubMed:17290226).
Joins single-strand breaks in a double-stranded polydeoxynucleotide in an ATP-dependent reaction (PubMed:9242410, PubMed:9809069, PubMed:12517771, PubMed:17290226).
LIG4 is mechanistically flexible: it can ligate nicks as well as compatible DNA overhangs alone, while in the presence of XRCC4, it can ligate ends with 2-nucleotides (nt) microhomology and 1-nt gaps (PubMed:17290226).
Forms a subcomplex with XRCC4; the LIG4-XRCC4 subcomplex is responsible for the NHEJ ligation step and XRCC4 enhances the joining activity of LIG4 (PubMed:9242410, PubMed:9809069).
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 (PubMed:10854421).
LIG4 regulates nuclear localization of XRCC4 (PubMed:24984242).
Biological Process
Cell cycleIEA:UniProtKB-KW
Cell divisionIEA:UniProtKB-KW
Cell population proliferationISS:UniProtKB
Cellular response to ionizing radiationManual Assertion Based On ExperimentIGI:UniProtKB
Cellular response to lithium ionIEA:Ensembl
Central nervous system developmentISS:UniProtKB
Chromosome organizationISS:UniProtKB
DNA biosynthetic processIEA:InterPro
DNA ligationManual Assertion Based On ExperimentIDA:UniProtKB
DNA ligation involved in DNA recombinationISS:UniProtKB
DNA ligation involved in DNA repairManual Assertion Based On ExperimentIDA:UniProtKB
DNA replicationIEA:UniProtKB-KW
Double-strand break repairManual Assertion Based On ExperimentIDA:UniProtKB
Double-strand break repair via classical nonhomologous end joiningManual Assertion Based On ExperimentIMP:BHF-UCL
Double-strand break repair via nonhomologous end joiningManual Assertion Based On ExperimentIDA:UniProtKB
Establishment of integrated proviral latencyTAS:Reactome
Immunoglobulin V(D)J recombinationManual Assertion Based On ExperimentIBA:GO_Central
In utero embryonic developmentISS:UniProtKB
Isotype switchingISS:UniProtKB
Negative regulation of neuron apoptotic processISS:UniProtKB
Neuron apoptotic processISS:UniProtKB
Nucleotide-excision repair, DNA gap fillingManual Assertion Based On ExperimentIDA:UniProtKB
Positive regulation of chromosome organizationManual Assertion Based On ExperimentIMP:BHF-UCL
Positive regulation of fibroblast proliferationISS:UniProtKB
Positive regulation of neurogenesisISS:UniProtKB
Pro-B cell differentiationISS:UniProtKB
Response to gamma radiationISS:UniProtKB
Response to X-rayManual Assertion Based On ExperimentIMP:UniProtKB
Single strand break repairManual Assertion Based On ExperimentIDA:UniProtKB
Somatic stem cell population maintenanceISS:UniProtKB
T cell differentiation in thymusISS:UniProtKB
T cell receptor V(D)J recombinationISS:UniProtKB
V(D)J recombinationManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Nucleus
Involvement in disease
LIG4 syndrome (LIG4S):
Characterized by immunodeficiency and developmental and growth delay. Patients display unusual facial features, microcephaly, growth and/or developmental delay, pancytopenia, and various skin abnormalities.
Severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-positive with sensitivity to ionizing radiation (RSSCID):
A form of severe combined immunodeficiency, 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 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.

Stinson, B. M., Carney, S. M., Walter, J. C., & Loparo, J. J. (2022). Structural role for DNA Ligase IV in promoting the fidelity of non-homologous end joining. BioRxiv, 2022-10.

Guerrero, E. N., Mitra, J., Wang, H., Rangaswamy, S., Hegde, P. M., Basu, P., ... & Hegde, M. L. (2019). Amyotrophic lateral sclerosis-associated TDP-43 mutation Q331K prevents nuclear translocation of XRCC4-DNA ligase 4 complex and is linked to genome damage-mediated neuronal apoptosis. Human Molecular Genetics, 28(15), 2459-2476.

Staines Boone, A. T., Chinn, I. K., Alaez-Versón, C., Yamazaki-Nakashimada, M. A., Carrillo-Sánchez, K., García-Cruz, M. D. L. L. H., ... & Lugo Reyes, S. O. (2019). Failing to make ends meet: the broad clinical Spectrum of DNA ligase IV deficiency. Case series and review of the literature. Frontiers in Pediatrics, 6, 426.

Schober, S., Schilbach, K., Doering, M., Cabanillas Stanchi, K. M., Holzer, U., Kasteleiner, P., ... & Handgretinger, R. (2019). Allogeneic hematopoietic stem cell transplantation in two brothers with DNA ligase IV deficiency: a case report and review of the literature. BMC pediatrics, 19(1), 1-10.

Kaminski, A. M., Tumbale, P. P., Schellenberg, M. J., Williams, R. S., Williams, J. G., Kunkel, T. A., ... & Bebenek, K. (2018). Structures of DNA-bound human ligase IV catalytic core reveal insights into substrate binding and catalysis. Nature communications, 9(1), 2642.

Ito, Y., Watanabe, T., Aikawa, S., Nishi, T., Nishiyama, T., Nakamura, Y., ... & Kondo, A. (2018). Deletion of DNA ligase IV homolog confers higher gene targeting efficiency on homologous recombination in Komagataella phaffii. FEMS yeast research, 18(7), foy074.

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For research use only. Not intended for any clinical use.

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