TRAF3IP1

Chr 2AR

TRAF3 interacting protein 1

Also known as: CFAP116, FAP116, IFT54, MIP-T3, MIPT3, SLSN9

The protein interacts with TNF receptor-associated factor 3, inhibits IL13 signaling, and regulates microtubule cytoskeleton organization and ciliogenesis. Mutations cause Senior-Loken syndrome 9, a ciliopathy affecting the kidneys and retina with autosomal recessive inheritance. The gene shows tolerance to loss-of-function variation (LOEUF 0.737), suggesting that complete loss of function may be required for disease manifestation.

Summary from RefSeq, OMIM, UniProt
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Primary Disease Associations & Inheritance

Senior-Loken syndrome 9MIM #616629
AR
0
Active trials
5
Pubs (1 yr)
22
P/LP submissions
0%
P/LP missense
0.74
LOEUF
DN
Mechanism· predicted
Clinical SummaryTRAF3IP1
🧬
Gene-Disease Validity (ClinGen)
ciliopathy · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
19 unique Pathogenic / Likely Pathogenic· 105 VUS of 200 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.74LOEUF
pLI 0.000
Z-score 2.83
OE 0.50 (0.350.74)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.33Z-score
OE missense 0.95 (0.871.04)
346 obs / 363.6 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.50 (0.350.74)
00.351.4
Missense OE0.95 (0.871.04)
00.61.4
Synonymous OE1.03
01.21.6
LoF obs/exp: 19 / 37.8Missense obs/exp: 346 / 363.6Syn Z: -0.30
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveTRAF3IP1-related Senior-Loken syndromeOTHERAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6842th %ile
GOF
0.3590th %ile
LOF
0.4038th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

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

ClinVar Variant Classifications

200 submitted variants in ClinVar

Classification Summary

Pathogenic12
Likely Pathogenic7
VUS105
Likely Benign41
Benign9
Conflicting2
12
Pathogenic
7
Likely Pathogenic
105
VUS
41
Likely Benign
9
Benign
2
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
6
0
6
0
12
Likely Pathogenic
7
0
0
0
7
VUS
1
96
8
0
105
Likely Benign
0
0
21
20
41
Benign
0
0
9
0
9
Conflicting
2
Total14964420176

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

TRAF3IP1 · 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
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