ARMC9

Chr 2AR

armadillo repeat containing 9

Also known as: ARM, JBTS30, KU-MEL-1, NS21

The ARMC9 protein is required for ciliogenesis, specifically regulating ciliary microtubule modifications and cilium length, and acts as a positive regulator of hedgehog signaling by participating in GLI2 and GLI3 protein trafficking at the ciliary tip. Biallelic mutations cause Joubert syndrome 30, inherited in an autosomal recessive pattern. This gene is not highly constrained against loss-of-function variants.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.051 OMIM phenotype
Clinical SummaryARMC9
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Gene-Disease Validity (ClinGen)
Joubert syndrome 30 · 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.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.05LOEUF
pLI 0.000
Z-score 1.29
OE 0.78 (0.581.05)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.85Z-score
OE missense 0.88 (0.800.96)
333 obs / 379.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.78 (0.581.05)
00.351.4
Missense OE0.88 (0.800.96)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 30 / 38.6Missense obs/exp: 333 / 379.7Syn Z: 0.19

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ARMC9 · 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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