WRAP53 RNA chaperone that plays a key role in telomere maintenance and RNA localization to Cajal bodies. Specifically recognizes and binds the Cajal body box (CAB box) present in both small Cajal body RNAs (scaRNAs) and telomerase RNA template component (TERC). Essential component of the telomerase holoenzyme complex, a ribonucleoprotein complex essential for the replication of chromosome termini that elongates telomeres in most eukaryotes. In the telomerase holoenzyme complex, required to stimulate the catalytic activity of the complex. 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. In addition, also controls telomerase holoenzyme complex localization to Cajal body. During S phase, required for delivery of TERC to telomeres during S phase and for telomerase activity. In addition to its role in telomere maintenance, also required for Cajal body formation, probably by mediating localization of scaRNAs to Cajal bodies. 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. Acts by recruiting the ubiquitin ligase RNF8 to DNA breaks and promote both homologous recombination (HR) and non-homologous end joining (NHEJ). Belongs to the TCAB1 family. Expressed in all tissues and cell lines examined. Note: This description may include information from UniProtKB.
Chromosomal Location of human Ortholog: 17p13.1
Cellular Component:  Cajal body; chromosome, telomeric region; cytosol; nuclear body; nucleoplasm; site of double-strand break; telomerase holoenzyme complex
Molecular Function:  chaperone binding; histone binding; identical protein binding; protein binding; protein-containing complex binding; RNA binding; telomerase RNA binding; ubiquitin protein ligase binding
Biological Process:  Cajal body organization; DNA repair; positive regulation of DNA repair; positive regulation of double-strand break repair; positive regulation of double-strand break repair via homologous recombination; positive regulation of double-strand break repair via nonhomologous end joining; positive regulation of establishment of protein localization to telomere; positive regulation of telomerase activity; protein localization to Cajal body; RNA folding; scaRNA localization to Cajal body; telomerase RNA localization to Cajal body; telomere formation via telomerase; telomere maintenance via telomerase
Disease: Dyskeratosis Congenita, Autosomal Recessive, 3
Reference #:  Q9BUR4 (UniProtKB)
Alt. Names/Synonyms: DKCB3; FLJ10385; TCAB1; Telomerase Cajal body protein 1; WAP53; WD repeat containing antisense to TP53; WD repeat containing, antisense to TP53; WD repeat domain 79; WD repeat-containing protein 79; WD repeat-containing protein WRAP53; WD40 protein Wrap53; WD40 repeat-containing protein antisense to TP53; WD40 repeat-containing protein antisense to TP53 gene; WD40 repeat-containing protein encoding RNA antisense to p53; WDR79; WRAP53; WRAP53beta
Gene Symbols: WRAP53
Molecular weight: 59,309 Da
Basal Isoelectric point: 4.46  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein