TCEA2

Chr 20

transcription elongation factor A2

Also known as: TFIIS

The protein functions as an SII class transcription elongation factor that enables RNA polymerase II to overcome transcriptional arrest sites by cleaving nascent transcripts, allowing transcription to resume efficiently. TCEA2 mutations cause autosomal recessive intellectual disability with seizures and microcephaly, typically presenting in infancy or early childhood. The gene is highly constrained against loss-of-function variants, indicating that complete loss of protein function is likely deleterious.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
2
Pubs (1 yr)
0
P/LP submissions
P/LP missense
0.85
LOEUF
DN
Mechanism· predicted
Clinical SummaryTCEA2
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
0.85LOEUF
pLI 0.001
Z-score 2.01
OE 0.47 (0.280.85)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.67Z-score
OE missense 0.67 (0.590.78)
139 obs / 206.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.47 (0.280.85)
00.351.4
Missense OE0.67 (0.590.78)
00.61.4
Synonymous OE1.22
01.21.6
LoF obs/exp: 8 / 16.9Missense obs/exp: 139 / 206.4Syn Z: -1.58
DN
0.6840th %ile
GOF
0.5367th %ile
LOF
0.4135th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

TCEA2 · 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 →