MAPK8IP3

Chr 16AD

mitogen-activated protein kinase 8 interacting protein 3

Also known as: JIP-3, JIP3, JSAP1, NEDBA, SYD2, syd

The protein functions as a scaffold protein that mediates JNK signaling and regulates axonal transport by bridging interactions between motor proteins like kinesin-1 and neuronal cargo, promoting axon elongation and cortical neuronal migration during brain development. Mutations cause neurodevelopmental disorder with or without variable brain abnormalities with autosomal dominant inheritance. This gene is highly constrained against loss-of-function mutations (pLI ~1.0, LOEUF 0.15), indicating that even single functional copies are critical for normal neurodevelopment.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismADLOEUF 0.151 OMIM phenotype
Clinical SummaryMAPK8IP3
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Gene-Disease Validity (ClinGen)
neurodevelopmental disorder with or without variable brain abnormalities; NEDBA · ADDefinitive

Definitive — sufficient evidence for diagnostic panels

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.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

LoF intolerant — likely haploinsufficient
LoF Constraint
0.15LOEUF
pLI 1.000
Z-score 7.19
OE 0.07 (0.040.15)
Highly constrained

Among the most LoF-intolerant genes (~top 3%)

Missense Constraint
2.88Z-score
OE missense 0.73 (0.690.78)
663 obs / 907.5 exp
Mild constraint

Moderately missense-constrained (top ~2.5%)

Observed / Expected Ratios
LoF OE0.07 (0.040.15)
00.351.4
Missense OE0.73 (0.690.78)
00.61.4
Synonymous OE1.16
01.21.6
LoF obs/exp: 5 / 69.9Missense obs/exp: 663 / 907.5Syn Z: -2.54
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongMAPK8IP3-related intellectual disability with variable brain anomaliesLOFAD
DN
0.4289th %ile
GOF
0.4085th %ile
LOF
0.74top 10%

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

LOFprediction above median · LOEUF 0.15

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MAPK8IP3 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

3D Protein StructureAlphaFold
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC