TUFT1

Chr 1AR

tuftelin 1

Also known as: WHSF

Tuftelin is an acidic protein involved in dental enamel mineralization and structural organization of the epidermis. Mutations cause woolly hair-skin fragility syndrome, inherited in an autosomal recessive pattern. The gene is highly constrained against loss-of-function variants (LOEUF 0.711), reflecting its importance in epidermal and dental development.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 0.711 OMIM phenotype
Clinical SummaryTUFT1
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
0.71LOEUF
pLI 0.000
Z-score 2.67
OE 0.43 (0.270.71)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.90Z-score
OE missense 0.83 (0.740.94)
186 obs / 223.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.43 (0.270.71)
00.351.4
Missense OE0.83 (0.740.94)
00.61.4
Synonymous OE0.85
01.21.6
LoF obs/exp: 11 / 25.6Missense obs/exp: 186 / 223.8Syn Z: 1.04
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedTUFT1-related woolly hair and superficial skin fragilityOTHERAR

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

DN
0.7033th %ile
GOF
0.6932th %ile
LOF
0.3258th %ile

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.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TUFT1 · 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.

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Clinical Literature
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