IFT88

Chr 13

intraflagellar transport 88

Also known as: D13S1056E, DAF19, TG737, TTC10, hTg737

The protein positively regulates primary cilium biogenesis and is involved in autophagy through trafficking of ATG16L and expansion of autophagic compartments. Mutations cause autosomal recessive ciliopathy syndromes including nephronophthisis and Bardet-Biedl syndrome, affecting the kidneys, retina, and other organ systems. The gene shows moderate constraint against loss-of-function variants (LOEUF 0.708).

OMIMResearchSummary from RefSeq, UniProt
LOFmechanismLOEUF 0.71
Clinical SummaryIFT88
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
2 unique Pathogenic / Likely Pathogenic· 203 VUS of 300 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.71LOEUF
pLI 0.000
Z-score 3.21
OE 0.51 (0.370.71)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.34Z-score
OE missense 0.95 (0.881.04)
394 obs / 413.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.51 (0.370.71)
00.351.4
Missense OE0.95 (0.881.04)
00.61.4
Synonymous OE0.89
01.21.6
LoF obs/exp: 25 / 49.3Missense obs/exp: 394 / 413.2Syn Z: 0.98
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedIFT88-related non-syndromic retinal degenerationLOFAR

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

DN
0.78top 25%
GOF
0.5465th %ile
LOF
0.2969th %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

300 submitted variants in ClinVar

Classification Summary

Pathogenic2
VUS203
Likely Benign89
Benign4
Conflicting2
2
Pathogenic
203
VUS
89
Likely Benign
4
Benign
2
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
2
0
2
Likely Pathogenic
0
0
0
0
0
VUS
17
166
18
2
203
Likely Benign
0
3
46
40
89
Benign
0
0
3
1
4
Conflicting
2
Total171696943300

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

View in ClinVar →

Protein Context — Lollipop Plot

IFT88 · 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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