TEX10

Chr 9

testis expressed 10

The TEX10 protein functions as a component of the Five Friends of Methylated CHTOP (5FMC) complex that regulates gene transcription through ZNF148 desumoylation and also participates in the PELP1 complex required for 28S rRNA maturation and pre-60S ribosomal subunit processing. Mutations in TEX10 cause autosomal recessive neurodevelopmental disorders through loss-of-function mechanisms. The gene is highly intolerant to loss-of-function variation, with a pLI score near 1.0 and very low LOEUF score of 0.163.

OMIMResearchSummary from RefSeq, UniProt, Mechanism
LOFmechanismLOEUF 0.16
Clinical SummaryTEX10
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 1.00). One damaged copy is likely sufficient to cause disease.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.16LOEUF
pLI 1.000
Z-score 5.99
OE 0.06 (0.030.16)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint
1.54Z-score
OE missense 0.80 (0.740.87)
384 obs / 478.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.06 (0.030.16)
00.351.4
Missense OE0.80 (0.740.87)
00.61.4
Synonymous OE0.85
01.21.6
LoF obs/exp: 3 / 47.6Missense obs/exp: 384 / 478.9Syn Z: 1.62
DN
0.3196th %ile
GOF
0.2796th %ile
LOF
0.74top 10%

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

LOFprediction above median · LOEUF 0.16

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

TEX10 · 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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