TCF12

Chr 15ADAR

transcription factor 12

Also known as: CRS3, HEB, HH26, HTF4, HsT17266, TCF-12, bHLHb20, p64

The protein is a basic helix-loop-helix transcription factor that recognizes E-box DNA sequences and regulates lineage-specific gene expression through heterodimerization with other bHLH proteins. Loss-of-function mutations cause craniosynostosis 3 and hypogonadotropic hypogonadism with or without anosmia through both autosomal dominant and autosomal recessive inheritance patterns. The protein is expressed in multiple tissues including skeletal muscle, thymus, and immune cells where it controls developmental gene expression programs.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismAD/ARLOEUF 0.372 OMIM phenotypes
Clinical SummaryTCF12
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Gene-Disease Validity (ClinGen)
TCF12-related craniosynostosis · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.68) — some intolerance to loss-of-function variants.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.37LOEUF
pLI 0.676
Z-score 4.58
OE 0.21 (0.120.37)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.89Z-score
OE missense 0.87 (0.800.95)
338 obs / 387.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.21 (0.120.37)
00.351.4
Missense OE0.87 (0.800.95)
00.61.4
Synonymous OE1.15
01.21.6
LoF obs/exp: 8 / 38.8Missense obs/exp: 338 / 387.3Syn Z: -1.40
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveTCF12-related neurodevelopmental disorder with coronal craniosynostosisLOFAD
DN
0.5476th %ile
GOF
0.2895th %ile
LOF
0.78top 5%

The highest-scoring mechanism for this gene is loss-of-function (haploinsufficiency).

LOFprediction above median · 1 literature citation · LOEUF 0.37

Literature Evidence

LOFMutations in TCF12, encoding a basic helix-loop-helix partner of TWIST1, are a frequent cause of coronal craniosynostosisPMID:23354436

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

TCF12 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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