GLIS2

GLIS family zinc finger 2

Tier: Gold Standard Ciliary Genes

Ensembl:
ENSG00000126603
UniProt:
Q9BZE0
OMIM:
608539
Synonyms:
NPHP7

Ciliogenesis screen results (5 screens)

  • Kim2016: Not Reported
  • Wheway et al. 2015 (siRNA) [siRNA]: Ciliogenesis Defect (z=-6.83) PMID:26167766
  • Breslow et al. 2018 (CRISPR) [CRISPR]: No Significant Effect PMID:29459680
  • Roosing et al. 2015 (siRNA) [siRNA]: No effect PMID:26595381
  • Kim et al. 2010 (siRNA) [siRNA]: Longer Cilia (Area per Cilia z=2.46) PMID:20393562

Ciliopathy associations

  • Nephronophthisis

References: PMID:17618285, PMID:23559409

Subcellular localization

basal body, nucleus

References: PMID:41005307, PMID:27181777, PMID:24069149

Functional category

  • Ciliary assembly/disassembly
  • Cell migration & adhesion
  • Transcription regulation

Function

Bifunctional transcriptional regulator, either as an activator and a repressor (11741991). Repressor of Hh signaling. Loss of function causes nephronophthisis-like phenotype (27181777). Involved in epithelial-to- mesenchymal transition, fibrosis and apoptosis. Together with BBS1, GLIS2 (also called NPHP7) is required for normal ciliary motility, responsible for body assymmetry (24069149).