IFT140

Chr 16ADAR

intraflagellar transport 140

Also known as: CED5, MZSDS, PKD9, RP80, SRTD9, WDTC2, c305C8.4, c380F5.1

The protein is a component of intraflagellar transport complex A, which is required for retrograde ciliary transport and proper development and function of ciliated cells. Mutations cause a spectrum of ciliopathies including cranioectodermal dysplasia, short-rib thoracic dysplasia with or without polydactyly, retinitis pigmentosa, and polycystic kidney disease. Inheritance is both autosomal dominant and autosomal recessive depending on the specific condition and variant.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismAD/ARLOEUF 0.824 OMIM phenotypes
Clinical SummaryIFT140
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Gene-Disease Validity (ClinGen)
IFT140-related recessive ciliopathy · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

2 total gene-disease associations curated

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.82LOEUF
pLI 0.000
Z-score 2.87
OE 0.64 (0.510.82)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.81Z-score
OE missense 1.07 (1.021.13)
997 obs / 927.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.64 (0.510.82)
00.351.4
Missense OE1.07 (1.021.13)
00.61.4
Synonymous OE1.14
01.21.6
LoF obs/exp: 49 / 76.0Missense obs/exp: 997 / 927.5Syn Z: -2.16
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveIFT140-related short-rib thoracic dysplasia with or without polydactylyLOFAR

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

DN
0.6261th %ile
GOF
0.4678th %ile
LOF
0.3551th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

IFT140 · 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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Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗