KIF4A

Chr XXLR

kinesin family member 4A

This protein is an ATP-dependent motor protein that transports membranous organelles along microtubules and plays essential roles in chromosome segregation and cytokinesis during cell division. Mutations cause X-linked intellectual developmental disorder and dental abnormalities including taurodontism, microdontia, and dens invaginatus. The gene follows X-linked recessive inheritance and is highly constrained against loss-of-function variants, indicating that such mutations are likely to be pathogenic.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismXLRLOEUF 0.202 OMIM phenotypes
Clinical SummaryKIF4A
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Gene-Disease Validity (ClinGen)
complex neurodevelopmental disorder with or without congenital anomalies · XLLimited

Limited evidence — not for standalone diagnostic reporting

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)

ncbi: Error: NCBI fetch failed: 429 https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esearch.fcgi

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.20LOEUF
pLI 1.000
Z-score 6.08
OE 0.10 (0.050.20)
Highly constrained

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

Missense Constraint
2.56Z-score
OE missense 0.66 (0.600.73)
300 obs / 453.3 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.10 (0.050.20)
00.351.4
Missense OE0.66 (0.600.73)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 5 / 52.5Missense obs/exp: 300 / 453.3Syn Z: 0.74

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KIF4A · 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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