ZNF24

Chr 18

zinc finger protein 24

Also known as: KOX17, RSG-A, ZNF191, ZSCAN3, Zfp191

This transcription factor is required for oligodendrocyte myelination and the conversion from premyelinating to myelinating states in the central nervous system. Mutations cause autosomal dominant neurodevelopmental disorders affecting white matter and myelination. The gene is highly constrained against loss-of-function variants (pLI 0.98, LOEUF 0.30), indicating that haploinsufficiency is likely not tolerated.

Summary from RefSeq, UniProt
Research Assistant →
0
Active trials
8
Pubs (1 yr)
45
P/LP submissions
0%
P/LP missense
0.30
LOEUF· LoF intol.
Mechanism
Clinical SummaryZNF24
Population Constraint (gnomAD)
Highly constrained gene — heterozygous loss-of-function variants are very rare in the population (pLI 0.98). One damaged copy is likely sufficient to cause disease.
📋
ClinVar Variants
43 unique Pathogenic / Likely Pathogenic· 30 VUS of 78 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.30LOEUF
pLI 0.981
Z-score 3.83
OE 0.10 (0.040.30)
Highly constrained

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

Missense Constraint
2.05Z-score
OE missense 0.59 (0.500.69)
114 obs / 194.3 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.10 (0.040.30)
00.351.4
Missense OE0.59 (0.500.69)
00.61.4
Synonymous OE0.91
01.21.6
LoF obs/exp: 2 / 20.9Missense obs/exp: 114 / 194.3Syn Z: 0.56

ClinVar Variant Classifications

78 submitted variants in ClinVar

Classification Summary

Pathogenic40
Likely Pathogenic3
VUS30
Likely Benign1
40
Pathogenic
3
Likely Pathogenic
30
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
40
0
40
Likely Pathogenic
0
0
3
0
3
VUS
0
29
1
0
30
Likely Benign
0
0
1
0
1
Benign
0
0
0
0
0
Total02945074

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

ZNF24 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →