IFT122

Chr 3AR

intraflagellar transport 122

Also known as: CED, CED1, CFAP80, FAP80, SPG, WDR10, WDR10p, WDR140

This gene encodes a member of the WD repeat protein family. WD repeats are minimally conserved regions of approximately 40 amino acids typically bracketed by gly-his and trp-asp (GH-WD), which may facilitate formation of heterotrimeric or multiprotein complexes. Members of this family are involved in a variety of cellular processes, including cell cycle progression, signal transduction, apoptosis, and gene regulation. This cytoplasmic protein contains seven WD repeats and an AF-2 domain which function by recruiting coregulatory molecules and in transcriptional activation. Mutations in this gene cause cranioectodermal dysplasia-1. A related pseudogene is located on chromosome 3. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2013]

GeneReviewsOMIMResearchGenerating clinical summary…
LOFmechanismARLOEUF 0.861 OMIM phenotype
Clinical SummaryIFT122
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Gene-Disease Validity (ClinGen)
cranioectodermal dysplasia 1 · 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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GeneReview available — IFT122
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.86LOEUF
pLI 0.000
Z-score 2.56
OE 0.68 (0.540.86)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.35Z-score
OE missense 0.96 (0.901.03)
689 obs / 715.0 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.68 (0.540.86)
00.351.4
Missense OE?0.96 (0.901.03)
00.61.4
Synonymous OE?0.97
01.21.6
LoF obs/exp: 49 / 72.5Missense obs/exp: 689 / 715.0Syn Z: 0.36

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

IFT122 · protein map & ClinVar variants

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

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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