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This gene encodes an F-actin-binding protein that cross-links actin to other cytoskeletal proteins, binds microtubules, regulates focal adhesion dynamics, and plays essential roles in neurite outgrowth and spine formation during brain development. Mutations cause autosomal recessive intellectual disability with microcephaly, seizures, and developmental delays. The protein's critical functions in cytoskeletal organization and neuronal development explain the severe neurodevelopmental phenotype observed in affected individuals.

ResearchSummary from UniProt
LOEUF 0.61
Clinical SummaryKIAA0754
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.29) despite low pLI — interpret in context.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.61LOEUF
pLI 0.085
Z-score 2.74
OE 0.29 (0.150.61)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.58Z-score
OE missense 0.93 (0.871.00)
586 obs / 627.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.29 (0.150.61)
00.351.4
Missense OE0.93 (0.871.00)
00.61.4
Synonymous OE0.97
01.21.6
LoF obs/exp: 5 / 17.3Missense obs/exp: 586 / 627.1Syn Z: 0.41

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KIAA0754 · 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 →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found