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Details on Person UniProt:Q9NZ71 RTEL1

Class:IdReferenceGeneProduct:96291
_chainChangeLogchain:1-1219 added on Sat February 7 2015
_displayNameUniProt:Q9NZ71 RTEL1
_timestamp2025-02-21 19:32:17
chainchain:1-1219
checksum28DFCFCC48BC0055
commentFUNCTION A probable ATP-dependent DNA helicase implicated in telomere-length regulation, DNA repair and the maintenance of genomic stability. Acts as an anti-recombinase to counteract toxic recombination and limit crossover during meiosis. Regulates meiotic recombination and crossover homeostasis by physically dissociating strand invasion events and thereby promotes noncrossover repair by meiotic synthesis dependent strand annealing (SDSA) as well as disassembly of D loop recombination intermediates. Also disassembles T loops and prevents telomere fragility by counteracting telomeric G4-DNA structures, which together ensure the dynamics and stability of the telomere.CATALYTIC ACTIVITY ATP + H2O = ADP + phosphate + H(+)SUBUNIT Interacts with TERF1 (PubMed:23959892). Interacts (via PIP-box) with PCNA; the interaction is direct and essential for suppressing telomere fragility. Interacts with MMS19; the interaction mediates the association of RTEL1 with the cytosolic iron-sulfur protein assembly (CIA) complex (PubMed:22678361, PubMed:23585563).INTERACTION Colocalizes with PCNA within the replication foci in S-phase cells.ALTERNATIVE PRODUCTS Additional isoforms seem to exist.DOMAIN The PIP-box (PCNA interacting peptide) motif mediates the interaction with PCNA and localization to replication foci.DISEASE The disease is caused by variants affecting the gene represented in this entry. RTEL1 mutations have also been found in patients with a dyskeratosis congenita-like phenotype consisting of one feature of dyskeratosis congenita and short telomeres, in the absence of the typical DKC diagnostic triad (PubMed:23329068).DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.MISCELLANEOUS Amplified in gastric tumors.MISCELLANEOUS Variant in position: 1264:R->H (in DKCB5), abolishes activity.SIMILARITY Belongs to the helicase family. RAD3/XPD subfamily.
descriptionrecommendedName: fullName evidence="1"Regulator of telomere elongation helicase 1 ecNumber evidence="1"5.6.2.- alternativeName: Novel helicase-like
geneNameRTEL1
C20orf41
KIAA1088
NHL
identifierQ9NZ71
isSequenceChangedFALSE
keyword3D-structure
4Fe-4S
Alternative splicing
ATP-binding
Disease variant
DNA damage
DNA repair
DNA-binding
Dyskeratosis congenita
Helicase
Hydrolase
Iron
Iron-sulfur
Isomerase
Metal-binding
Nucleotide-binding
Nucleus
Proteomics identification
Reference proteome
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9862192] Weiser, Joel, 2024-02-26
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9939033] Weiser, Joel, 2025-02-21
nameRTEL1
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8995106] ENSEMBL:ENSG00000258366 RTEL1 [Homo sapiens]
secondaryIdentifierRTEL1_HUMAN
A2A397
A2A398
B4DRM5
B4DYM3
B4E3N6
E1P5J4
E1P5J5
Q5JTV3
Q5JTV4
Q9BW37
Q9H402
Q9H4X6
Q9NX25
Q9NZ73
Q9UPR4
Q9Y4R6
sequenceLength1219
species[Species:48887] Homo sapiens
(isoformParent)[ReferenceIsoform:151045] UniProt:Q9NZ71-2 RTEL1 [Homo sapiens]
[ReferenceIsoform:151047] UniProt:Q9NZ71-4 RTEL1 [Homo sapiens]
[ReferenceIsoform:246723] UniProt:Q9NZ71-5 RTEL1 [Homo sapiens]
[ReferenceIsoform:414935] UniProt:Q9NZ71-1 RTEL1 [Homo sapiens]
[ReferenceIsoform:424710] UniProt:Q9NZ71-6 RTEL1 [Homo sapiens]
[ReferenceIsoform:424711] UniProt:Q9NZ71-7 RTEL1 [Homo sapiens]
[ReferenceIsoform:424712] UniProt:Q9NZ71-8 RTEL1 [Homo sapiens]
[ReferenceIsoform:424713] UniProt:Q9NZ71-9 RTEL1 [Homo sapiens]
(referenceEntity)[EntityWithAccessionedSequence:2564769] RTEL1 [cytosol] [Homo sapiens]
[EntityWithAccessionedSequence:5686112] RTEL1 [nucleoplasm] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:Q9NZ71 RTEL1 (96291)