KLF7

Chr 2

KLF transcription factor 7

Also known as: UKLF

KLF7 encodes a transcriptional factor containing C2H2 zinc fingers that plays a critical role in neuronal morphogenesis and survival of sensory neurons, while also regulating insulin sensitivity and adipogenesis. Mutations cause autosomal dominant developmental delay with variable intellectual disability and dysmorphic facies. This gene is highly constrained against loss-of-function variants, indicating that such mutations are likely to be pathogenic.

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.24
Clinical SummaryKLF7
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
11 unique Pathogenic / Likely Pathogenic of 11 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.24LOEUF
pLI 0.982
Z-score 3.24
OE 0.00 (0.000.24)
Highly constrained

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

Missense Constraint
2.30Z-score
OE missense 0.53 (0.450.63)
101 obs / 190.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.00 (0.000.24)
00.351.4
Missense OE0.53 (0.450.63)
00.61.4
Synonymous OE1.14
01.21.6
LoF obs/exp: 0 / 12.2Missense obs/exp: 101 / 190.5Syn Z: -1.01
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
moderateKLF7-related developmental disorderOTHERAD
DN
0.3892th %ile
GOF
0.2994th %ile
LOF
0.83top 5%

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

LOFprediction above median · LOEUF 0.24

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

11 submitted variants in ClinVar

Classification Summary

Pathogenic11
11
Pathogenic

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
11
Likely Pathogenic
0
VUS
0
Likely Benign
0
Benign
0
Total11

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

KLF7 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →