TP73

Chr 1AR

tumor protein p73

Also known as: CILD47, P73

This protein is a p53 family transcription factor that participates in apoptotic responses to DNA damage and serves as an essential regulator of ciliated cell differentiation in the lungs and other tissues. Mutations cause autosomal recessive primary ciliary dyskinesia and lissencephaly, affecting the respiratory system and brain development. The gene is highly constrained against loss-of-function variants (pLI 0.997, LOEUF 0.26), reflecting its critical developmental importance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.261 OMIM phenotype
Clinical SummaryTP73
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Gene-Disease Validity (ClinGen)
ciliary dyskinesia, primary, 47, and lissencephaly · ARStrong

Strong evidence — appropriate for clinical testing

Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.26LOEUF
pLI 0.997
Z-score 4.59
OE 0.10 (0.040.26)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
1.85Z-score
OE missense 0.75 (0.680.82)
322 obs / 430.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.10 (0.040.26)
00.351.4
Missense OE0.75 (0.680.82)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 3 / 30.3Missense obs/exp: 322 / 430.1Syn Z: -1.17
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongTP73-related ciliary dyskinesia and lissencephalyLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.4389th %ile
GOF
0.3491th %ile
LOF
0.76top 10%

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312. Mechanism ranking also informed by gnomAD constraint, ClinVar, and ClinGen data.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TP73 · 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 →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗