TELO2

Chr 16AR

telomere maintenance 2

TELO2 encodes a protein that regulates the DNA damage response and is required to stabilize phosphatidylinositol 3-kinase-related protein kinases, while also promoting assembly and maintaining activity of mTORC1 and mTORC2 complexes that regulate cell growth and survival. Mutations cause You-Hoover-Fong syndrome, which follows autosomal recessive inheritance. The gene shows minimal constraint against loss-of-function variants, consistent with its recessive disease mechanism.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 0.931 OMIM phenotype
Clinical SummaryTELO2
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Gene-Disease Validity (ClinGen)
TELO2-related intellectual disability-neurodevelopmental disorder · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.93LOEUF
pLI 0.000
Z-score 1.93
OE 0.67 (0.490.93)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.37Z-score
OE missense 1.04 (0.971.12)
568 obs / 543.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.67 (0.490.93)
00.351.4
Missense OE1.04 (0.971.12)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 27 / 40.2Missense obs/exp: 568 / 543.9Syn Z: -1.95

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TELO2 · 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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