Reactome: A Curated Pathway Database
THIS SITE IS USED FOR CURATION AND TESTING
IT IS NOT STABLE, IS LINKED TO AN INCOMPLETE DATA SET, AND IS NOT MONITORED FOR PERFORMANCE. WE STRONGLY RECOMMEND THE USE OF OUR PUBLIC SITE

Query author contributions in Reactome

Reactome depends on collaboration between our curation team and outside experts to assemble and peer-review its pathway modules. The integration of ORCID within Reactome enables us to meet a key challenge with authoring, curating and reviewing biological information by incentivizing and crediting the external experts that contribute their expertise and time to the Reactome curation process. More information is available at ORCID and Reactome.

If you have an ORCID ID that is not listed on this page, please forward this information to us and we will update your Reactome pathway records.

Name Email address

Details on Person UniProt:Q9BUR4 WRAP53

Class:IdReferenceGeneProduct:254682
_chainChangeLogchain:1-548 added on Fri February 6 2015
_displayNameUniProt:Q9BUR4 WRAP53
_timestamp2024-11-03 20:08:01
chainchain:1-548
checksum4687517268A813B5
commentFUNCTION RNA chaperone that plays a key role in telomere maintenance and RNA localization to Cajal bodies (PubMed:29695869, PubMed:29804836). 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:29695869, PubMed:29804836). 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).SUBUNIT Component of the telomerase holoenzyme complex composed of one molecule of TERT, one molecule of WRAP53/TCAB1, two molecules of H/ACA ribonucleoprotein complex subunits DKC1, NOP10, NHP2 and GAR1, and a telomerase RNA template component (TERC) (PubMed:19179534, PubMed:20351177, PubMed:26170453, PubMed:29695869). The telomerase holoenzyme complex is associated with TEP1, SMG6/EST1A and POT1 (PubMed:19179534). Interacts with the chaperonin-containing T-complex (TRiC) complex; which mediates the folding of WRAP53/TCAB1 (PubMed:25467444). Interacts with COIL (PubMed:21072240). Interacts with SMN1 (PubMed:21072240). Interacts with RNF8 (PubMed:25512560). Interacts with histone H2AX (PubMed:26734725, PubMed:27715493).INTERACTION 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).TISSUE SPECIFICITY Expressed in all tissues and cell lines examined.INDUCTION (Microbial infection) Over-expressed following infection by Epstein-Barr virus.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.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS The mRNA encoding this protein plays a critical role in the regulation of p53/TP53 expression at the post-transcriptional level; it is involved both in maintaining basal p53/TP53 mRNA levels and in p53/TP53 induction upon DNA damage.SIMILARITY Belongs to the TCAB1 family.
created[InstanceEdit:217385] Schmidt, EE, 2008-03-27 06:23:53
descriptionrecommendedName: fullName evidence="22"Telomerase Cajal body protein 1 alternativeName: fullName evidence="24"WD repeat-containing protein 79 alternativeName: fullName evidence="23"WD40 repeat-containing protein antisense to TP53 gene shortName evidence="25"WRAP53beta
geneNameWRAP53
TCAB1
WDR79
identifierQ9BUR4
isSequenceChangedFALSE
keyword3D-structure
Chaperone
Chromosome
Disease variant
DNA damage
DNA repair
Dyskeratosis congenita
Host-virus interaction
Nucleus
Phosphoprotein
Proteomics identification
Reference proteome
Repeat
RNA-binding
Telomere
WD repeat
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9917590] Weiser, Joel, 2024-08-09
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
nameWRAP53
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8988208] ENSEMBL:ENSG00000141499 WRAP53 [Homo sapiens]
secondaryIdentifierTCAB1_HUMAN
B3KPR9
D3DTQ4
Q08ET9
Q9NW09
sequenceLength548
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:418386] WRAP53 [nucleoplasm] [Homo sapiens]
[EntityWithAccessionedSequence:6813830] Unfolded WRAP53 [cytosol] [Homo sapiens]
[Change default viewing format]
No pathways have been reviewed or authored by UniProt:Q9BUR4 WRAP53 (254682)