TMEFF2

Chr 2

transmembrane protein with EGF like and two follistatin like domains 2

Also known as: CT120.2, HPP1, TENB2, TPEF, TR, TR-2

This gene encodes a transmembrane protein from the tomoregulin family that functions as a survival factor for hippocampal and mesencephalic neurons and can regulate cell proliferation through ERK1/2 signaling. The gene is not well-established in pediatric neurogenetic disorders, with constraint metrics (pLI 0.00007, LOEUF 0.739) indicating tolerance to loss-of-function variants. Current evidence primarily links this gene to cancer biology rather than inherited neurological conditions.

OMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 0.74
Clinical SummaryTMEFF2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.74LOEUF
pLI 0.000
Z-score 2.54
OE 0.45 (0.280.74)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.60Z-score
OE missense 0.69 (0.600.79)
140 obs / 204.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.45 (0.280.74)
00.351.4
Missense OE0.69 (0.600.79)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 11 / 24.6Missense obs/exp: 140 / 204.4Syn Z: 0.79
DN
0.7131th %ile
GOF
0.7126th %ile
LOF
0.3162th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TMEFF2 · 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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