TOE1

Chr 1AR

target of EGR1, exonuclease

Also known as: PCH7, TOE-1, hCaf1z

TOE1 encodes a poly(A)-specific ribonuclease that processes snRNA 3'-ends and regulates cell growth by inducing CDKN1A and TGF-beta expression. Mutations cause pontocerebellar hypoplasia type 7, an autosomal recessive disorder characterized by severe underdevelopment of the pons and cerebellum. The gene shows extreme intolerance to loss-of-function variants (pLI near 1), indicating that complete loss of function is likely incompatible with normal development.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.461 OMIM phenotype
Clinical SummaryTOE1
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
1.46LOEUF
pLI 0.000
Z-score -0.13
OE 1.03 (0.741.46)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
1.07Z-score
OE missense 0.83 (0.740.92)
246 obs / 297.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.03 (0.741.46)
00.351.4
Missense OE0.83 (0.740.92)
00.61.4
Synonymous OE0.99
01.21.6
LoF obs/exp: 23 / 22.4Missense obs/exp: 246 / 297.8Syn Z: 0.12

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TOE1 · 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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