IFT27

Chr 22AR

intraflagellar transport 27

Also known as: BBS19, CFAP156, FAP156, RABL4, RAYL

This gene encodes a small GTPase component of the intraflagellar transport complex B that promotes exit of the BBSome complex from cilia and is required for hedgehog signaling. Mutations cause Bardet-Biedl syndrome 19, a ciliopathy affecting multiple organ systems including the kidneys, retina, and reproductive system. The condition follows autosomal recessive inheritance.

OMIMResearchSummary from RefSeq, OMIM, UniProt
MultiplemechanismARLOEUF 1.411 OMIM phenotype
Clinical SummaryIFT27
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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.
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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

Tolerant — LoF & missense variants common in population
LoF Constraint
1.41LOEUF
pLI 0.000
Z-score 0.58
OE 0.81 (0.491.41)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.22Z-score
OE missense 0.94 (0.801.11)
98 obs / 104.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.81 (0.491.41)
00.351.4
Missense OE0.94 (0.801.11)
00.61.4
Synonymous OE0.86
01.21.6
LoF obs/exp: 9 / 11.1Missense obs/exp: 98 / 104.2Syn Z: 0.73
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
limitedIFT27-related Bardet-Biedl syndromeOTHERAR

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

DN
0.80top 25%
GOF
0.82top 10%
LOF
0.2190th %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

IFT27 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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