SIX3

SIX homeobox 3

Ensembl:
ENSG00000138083
UniProt:
O95343
OMIM:
603714
Synonyms:
HPE2

Cilia effects upon perturbation of SIX3

Ciliogenesis screen results (4 screens)

  • Wheway et al. 2015 (siRNA) [siRNA]: No effect PMID:26167766
  • Breslow et al. 2018 (CRISPR) [CRISPR]: No Significant Effect PMID:29459680
  • Roosing et al. 2015 (siRNA) [siRNA]: No effect PMID:26595381
  • Elliott et al. 2025 (CRISPRa) [CRISPRa]: Disassembly Trigger (casTLE Effect=-3.19) PMID:41160700

Ciliopathy associations

  • Holoprosencephaly

Subcellular localization

cilia associated gene, nucleus

Functional category

  • Ciliary assembly/disassembly
  • Protein processing & maturation
  • Transcription regulation

Function

Six3 and Six6 together suppress Wnt/β-catenin sig ling and Wnt3a/β-catenin sig ling promotes ciliary margin cells at the cost of neuroreti ( 30485816).

Model organism evidence

Mus musculus (1 reference)

In the absence of ARL13B, photoreceptors failed to develop outer segment (OS) membranous discs and axonemes, resulting in loss of function and rapid degeneration.

PMIDs: 30573647