WRAP53

Chr 17AR

WD repeat containing antisense to TP53

Also known as: DKCB3, TCAB1, WDR79

This RNA chaperone is an essential component of the telomerase holoenzyme complex required for telomere synthesis and also plays key roles in Cajal body formation and DNA repair. Mutations cause dyskeratosis congenita, autosomal recessive type 3, a disorder affecting multiple organ systems including the skin, nails, oral mucosa, bone marrow, and lungs. The gene shows high constraint against loss-of-function variants (pLI near 0, LOEUF 0.69) and follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.691 OMIM phenotype
Clinical SummaryWRAP53
🧬
Gene-Disease Validity (ClinGen)
dyskeratosis congenita · ARModerate

Moderate evidence — consider for supplementary testing

3 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.69LOEUF
pLI 0.000
Z-score 2.82
OE 0.43 (0.270.69)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.02Z-score
OE missense 1.00 (0.911.09)
315 obs / 316.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.43 (0.270.69)
00.351.4
Missense OE1.00 (0.911.09)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 12 / 28.2Missense obs/exp: 315 / 316.0Syn Z: -0.08

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

WRAP53 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →