EHBP1L1

EH domain binding protein 1 like 1

Ensembl:
ENSG00000173442
UniProt:
Q8N3D4
OMIM:
619583
Synonyms:
DKFZP762C186, TANGERIN

Cilia effects upon perturbation of EHBP1L1

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]: No Significant Effect PMID:29459680

Phenotypes

Mouse phenotype:
preweaning lethality, complete penetrance
Human ciliopathy phenotype:
Non-immune hydrops fetalis

Subcellular localization

cilia

Functional category

  • Ciliary assembly/disassembly
  • Actin & cytoskeleton regulation

Function

EHBP1L1 and binding proteins CD2AP and CIN85 localize to the ciliary sheath membrane, which is a subdomain of the CV, and negatively regulate ciliary length. Depletion of EHBP1L1 and CD2P/CIN85 causes actin nucleation and branching defects around the basal body, suggesting that actin network formation is crucial for maintaining an appropriate length of primary cilia(36754282).