KIAA0319L

Chr 1

KIAA0319 like

Also known as: AAVR, AAVRL

The protein is predicted to be involved in neuron migration and may play a role in axon guidance through interaction with RTN4R. This gene has not been definitively associated with human disease, though it is highly constrained against loss-of-function variants (LOEUF 0.449), suggesting it may be essential for normal development. Further research is needed to establish any clinical phenotypes associated with KIAA0319L variants.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.45
Clinical SummaryKIAA0319L
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.30) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.45LOEUF
pLI 0.001
Z-score 4.81
OE 0.30 (0.200.45)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.56Z-score
OE missense 0.81 (0.750.88)
455 obs / 558.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.30 (0.200.45)
00.351.4
Missense OE0.81 (0.750.88)
00.61.4
Synonymous OE0.84
01.21.6
LoF obs/exp: 16 / 54.2Missense obs/exp: 455 / 558.9Syn Z: 1.88
DN
0.6938th %ile
GOF
0.6247th %ile
LOF
0.3260th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KIAA0319L · 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 →