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Mouse Anti-WRAP53 Recombinant Antibody (CBWJW-017) (CBMAB-W0174-WJ)

This product is a mouse antibody that recognizes WRAP53. The antibody CBWJW-017 can be used for immunoassay techniques such as: WB, ELISA.
See all WRAP53 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBWJW-017
Antibody Isotype
IgG2a
Application
WB, ELISA

Basic Information

Specificity
Human
Antibody Isotype
IgG2a
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!]

Concentration
1 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
WD Repeat Containing Antisense To TP53
Introduction
WRAP53 is a protein-coding gene encodes an essential component of the telomerase holoenzyme complex which is a ribonucleoprotein complex required for telomere synthesis. Diseases associated with WRAP53 include Dyskeratosis Congenita, Autosomal Recessive 3 and Dyskeratosis Congenita. Among its related pathways are Chaperonin-mediated protein folding and Chromosome Maintenance.
Entrez Gene ID
55135
UniProt ID
Q9BUR4
Alternative Names
DKCB3; TCAB1; WDR79
Function
RNA chaperone that plays a key role in telomere maintenance and RNA localization to Cajal bodies (PubMed:29804836, PubMed:29695869).
Specifically recognizes and binds the Cajal body box (CAB box) present in both small Cajal body RNAs (scaRNAs) and telomerase RNA template component (TERC) (PubMed:19285445, PubMed:20351177, PubMed:29804836, PubMed:29695869).
Essential component of the telomerase holoenzyme complex, a ribonucleoprotein complex essential for the replication of chromosome termini that elongates telomeres in most eukaryotes (PubMed:19179534, PubMed:20351177, PubMed:26170453, PubMed:29695869).
In the telomerase holoenzyme complex, required to stimulate the catalytic activity of the complex (PubMed:27525486, PubMed:29804836).
Acts by specifically binding the CAB box of the TERC RNA and controlling the folding of the CR4/CR5 region of the TERC RNA, a critical step for telomerase activity (PubMed:29804836).
In addition, also controls telomerase holoenzyme complex localization to Cajal body (PubMed:22547674).
During S phase, required for delivery of TERC to telomeres during S phase and for telomerase activity (PubMed:29804836).
In addition to its role in telomere maintenance, also required for Cajal body formation, probably by mediating localization of scaRNAs to Cajal bodies (PubMed:19285445, PubMed:21072240).
Also plays a role in DNA repair: phosphorylated by ATM in response to DNA damage and relocalizes to sites of DNA double-strand breaks to promote the repair of DNA double-strand breaks (PubMed:25512560, PubMed:27715493).
Acts by recruiting the ubiquitin ligase RNF8 to DNA breaks and promote both homologous recombination (HR) and non-homologous end joining (NHEJ) (PubMed:25512560, PubMed:27715493).
Biological Process
Biological Process Cajal body organization Source:UniProtKB1 Publication
Biological Process DNA repair Source:UniProtKB-KW
Biological Process positive regulation of DNA repair Source:UniProtKB1 Publication
Biological Process positive regulation of double-strand break repair Source:UniProtKB1 Publication
Biological Process positive regulation of double-strand break repair via homologous recombination Source:UniProtKB1 Publication
Biological Process positive regulation of double-strand break repair via nonhomologous end joining Source:UniProtKB1 Publication
Biological Process positive regulation of establishment of protein localization to telomere Source:BHF-UCL1 Publication
Biological Process positive regulation of telomerase activity Source:UniProtKB2 Publications
Biological Process protein localization to Cajal body Source:UniProtKB1 Publication
Biological Process RNA folding Source:UniProtKB1 Publication
Biological Process scaRNA localization to Cajal body Source:UniProtKB1 Publication
Biological Process telomerase RNA localization to Cajal body Source:BHF-UCL1 Publication
Biological Process telomere formation via telomerase Source:UniProtKB1 Publication
Biological Process telomere maintenance via telomerase Source:UniProtKB2 Publications
Cellular Location
Nucleus, Cajal body
Chromosome, telomere
Chromosome
Released from telomerase RNA template component (TERC) in mitotic cells coincident with delocalization from Cajal bodies (PubMed:26170453).
In response to DNA damage, localizes to sites of DNA double-strand breaks following phosphorylation by ATM (PubMed:26734725, PubMed:27715493).
Involvement in disease
Dyskeratosis congenita, autosomal recessive, 3 (DKCB3):
A rare multisystem disorder caused by defective telomere maintenance. It is characterized by progressive bone marrow failure, and the clinical triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Common but variable features include premature graying, aplastic anemia, low platelets, osteoporosis, pulmonary fibrosis, and liver fibrosis among others. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy.
PTM
Phosphorylated at Ser-64 by ATM in response to DNA damage, promoting its interaction with histone H2AX and localization to sites of DNA double-strand breaks.
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For research use only. Not intended for any clinical use.

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