Rabbit Anti-DCLRE1C Recombinant Antibody (EG932) (V2LY-0125-LY1205)

Name: Rabbit Anti-DCLRE1C Recombinant Antibody (EG932)
SKU: V2LY-0125-LY1205
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Rabbit

Clone

EG932

Application

IF, WB

Immunogen

Recombinant protein encompassing a sequence within the center region of human Artemis.

Host Species

Rabbit

Specificity

Human

Antibody Isotype

IgG

Clonality

Monoclonal Antibody

Application Notes

Application	Note
WB	1:500-1:3,000

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

Format

Liquid

Buffer

PBS

Preservative

None

Concentration

1 mg/mL

Purity

>95% as determined by analysis by SDS-PAGE

Storage

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

More Infomation

Target

Full Name

DNA Cross-Link Repair 1C

Entrez Gene ID

64421

UniProt ID

Q96SD1

Function

Required for V(D)J recombination, the process by which exons encoding the antigen-binding domains of immunoglobulins and T-cell receptor proteins are assembled from individual V, (D), and J gene segments. V(D)J recombination is initiated by the lymphoid specific RAG endonuclease complex, which generates site specific DNA double strand breaks (DSBs). These DSBs present two types of DNA end structures: hairpin sealed coding ends and phosphorylated blunt signal ends. These ends are independently repaired by the non homologous end joining (NHEJ) pathway to form coding and signal joints respectively. This protein exhibits single-strand specific 5'-3' exonuclease activity in isolation and acquires endonucleolytic activity on 5' and 3' hairpins and overhangs when in a complex with PRKDC. The latter activity is required specifically for the resolution of closed hairpins prior to the formation of the coding joint. May also be required for the repair of complex DSBs induced by ionizing radiation, which require substantial end-processing prior to religation by NHEJ.

Biological Process

Adaptive immune response Source: UniProtKB-KW
B cell differentiation Source: Ensembl
Double-strand break repair via nonhomologous end joining Source: GO_Central
Interstrand cross-link repair Source: GO_Central
Protection from non-homologous end joining at telomere Source: GO_Central
Response to ionizing radiation Source: Ensembl
V(D)J recombination Source: Ensembl

Cellular Location

Nucleus

Involvement in disease

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.
Severe combined immunodeficiency Athabaskan type (SCIDA):
A variety of SCID with sensitivity to ionizing radiation. A founder mutation has been detected in Athabascan-speaking native Americans, being inherited as an autosomal recessive trait. Affected individuals exhibit clinical symptoms and defects in DNA repair comparable to those seen in RS-SCID.
Omenn syndrome (OS):
Severe immunodeficiency characterized by the presence of activated, anergic, oligoclonal T-cells, hypereosinophilia, and high IgE levels.

PTM

Phosphorylation on undefined residues by PRKDC may stimulate endonucleolytic activity on 5' and 3' hairpins and overhangs. PRKDC must remain present, even after phosphorylation, for efficient hairpin opening. Also phosphorylated by ATM in response to ionizing radiation (IR) and by ATR in response to ultraviolet (UV) radiation.

Strubbe, S., De Bruyne, M., Pannicke, U., Beyls, E., Vandekerckhove, B., Leclercq, G., ... & Taghon, T. (2021). A Novel Non-Coding Variant in DCLRE1C Results in Deregulated Splicing and Induces SCID Through the Generation of a Truncated ARTEMIS Protein That Fails to Support V (D) J Recombination and DNA Damage Repair. Frontiers in immunology, 12, 2398.

Li, Y., Adur, M. K., Wang, W., Schultz, R. B., Hale, B., Wierson, W., ... & Ross, J. W. (2021). Effect of ARTEMIS (DCLRE1C) deficiency and microinjection timing on editing efficiency during somatic cell nuclear transfer and in vitro fertilization using the CRISPR/Cas9 system. Theriogenology, 170, 107-116.

Mou, W., Gao, L., He, J., Yin, J., Xu, B., & Gui, J. (2021). Compound heterozygous DCLRE1C mutations lead to clinically typical Severe Combined Immunodeficiency presenting with Graft Versus Host Disease. Immunogenetics, 73(6), 425-434.

Rajaei, A. A., RASHIDINEZHAD, A., Alebouyeh, M., & Shirkoohi, R. (2021). Examining of relative alternations in the expression of CHEK2, DCLRE1C and XRCC4 genes in patients with Gastritis.

Nahum, A., Somech, R., Shubinsky, G., Levy, J., & Broides, A. (2020). Unusual phenotype in patients with a hypomorphic mutation in the DCLRE1C gene: IgG hypergammaglobulinemia with IgA and IgE deficiency. Clinical Immunology, 213, 108366.

Choe, N., Brick, L., Kozenko, M., Chakraborty, P., Kernohan, K. D., Bulman, D., & Brager, R. (2020). Positive newborn screen: a case of a novel variant in DCLRE1C in a patient with SCID. LymphoSign Journal, 7(1), 46-48.

Sundin, M., Marits, P., Ramme, K., Kolios, A. G., & Nilsson, J. (2019). Severe combined immunodeficiency (SCID) presenting in childhood, with agammaglobulinemia, associated with novel compound heterozygous mutations in DCLRE1C. Clinical Immunology, 200, 16-18.

Wu, Z., Subramanian, N., Jacobsen, E. M., Laib Sampaio, K., van der Merwe, J., Hönig, M., & Mertens, T. (2019). NK cells from RAG-or DCLRE1C-deficient patients inhibit HCMV. Microorganisms, 7(11), 546.

Sundin, M., Uhlin, M., Gaballa, A., Ramme, K., Kolios, A. G., Marits, P., & Nilsson, J. (2018). Late presenting atypical severe combined immunodeficiency (SCID) associated with a novel missense mutation in DCLRE1C. Pediatric Allergy and Immunology, 29(1), 108-111.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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

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