ZNF488

Chr 10

zinc finger protein 488

ZNF488 encodes a transcriptional repressor that promotes oligodendrocyte differentiation and contributes to remyelination following nerve injury by mediating Notch signaling pathways. Mutations cause autosomal recessive intellectual disability with white matter abnormalities, affecting central nervous system development and myelination. This gene is highly constrained against loss-of-function variants, indicating that complete loss of protein function is likely pathogenic.

Summary from RefSeq, UniProt
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0
Active trials
2
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.28
LOEUF
LOF
Mechanism· predicted
Clinical SummaryZNF488
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
1.28LOEUF
pLI 0.000
Z-score 0.83
OE 0.75 (0.461.28)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.14Z-score
OE missense 1.03 (0.921.15)
215 obs / 209.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.75 (0.461.28)
00.351.4
Missense OE1.03 (0.921.15)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 10 / 13.3Missense obs/exp: 215 / 209.2Syn Z: 0.18
DN
0.5968th %ile
GOF
0.3887th %ile
LOF
0.65top 25%

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

LOFprediction above median

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ZNF488 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

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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