ARMC9

armadillo repeat containing 9

Ensembl:
ENSG00000135931
UniProt:
Q7Z3E5
OMIM:
617612
Synonyms:
ARM, FLJ12584, KIAA1868, KU-MEL-1

Cilia effects upon perturbation of ARMC9

Loss-of-function effect:
Longer cilia
Overexpression effect:
No effect

Ciliogenesis screen results (2 screens)

  • Wheway et al. 2015 (siRNA) [siRNA]: No effect PMID:26167766
  • Breslow et al. 2018 (CRISPR) [CRISPR]: Positive Regulator (Hh signaling, casTLE effect=-2.3) PMID:29459680

Phenotypes

Human ciliopathy phenotype:
Joubert syndrome; Joubert syndrome 30

Ciliopathy associations

  • Joubert Syndrome

Subcellular localization

basal body, cilia

Functional category

  • Ciliary assembly/disassembly

Function

Ciliopathy gene JBTS30 (PMID:28625504). Interacts with coiled-coil domain containing 66 (CCDC66) and TOG array regulator of axonemal 1 (TOGARAM1) (32453716). Regulator of b- tubule length (30217954).Loss of function andof GFP-ARMC9B increased length of cilia, and overexpression of GFP-ARMC9B elongated distal segment (~30%), but effect was on geometry rather than whole cilium length(PMID: 30217954).

Model organism evidence

Danio rerio (2 references)

Mutations in ARMC9, which encodes a basal body protein, cause Joubert syndrome, a ciliopathy with defects in the brain, kidney, and eye.

Mutations in ARMC9, which Encodes a Basal Body Protein, Cause Joubert Syndrome in Humans and Ciliopathy Phenotypes in Zebrafish.

PMIDs: 38949024, 28625504