LZTFL1

leucine zipper transcription factor like 1

Ensembl:
ENSG00000163818
UniProt:
Q9NQ48
OMIM:
606568
Synonyms:
BBS17

Cilia effects upon perturbation of LZTFL1

Cilia number / % ciliated:
Decreased cilia number

Ciliogenesis screen results (3 screens)

  • Wheway et al. 2015 (siRNA) [siRNA]: No effect PMID:26167766
  • Breslow et al. 2018 (CRISPR) [CRISPR]: Positive Regulator (Hh signaling, casTLE effect=-2.94) PMID:29459680
  • Roosing et al. 2015 (siRNA) [siRNA]: No effect PMID:26595381

Phenotypes

Human ciliopathy phenotype:
Bardet-Biedl syndrome

Ciliopathy associations

  • Bardet-Biedl Syndrome

Subcellular localization

cilia, transition zone

Functional category

  • Ciliary assembly/disassembly
  • Trafficking (BBSome, small GTPases, vesicular transport, ATPases)
  • Actin & cytoskeleton regulation
  • T cell biology
  • Reproduction & sperm
  • Viral interactions
  • Cell migration & adhesion
  • Signaling (Hedgehog, GPCRs, ion channels)
  • Ciliary membrane
  • Transition zone
  • Transcription regulation
  • Cilia length regulation

Function

Interacts with a BBS protein complex known as the BBSome to negatively regulate ciliary trafficking of this complex inhibiting BBSome ciliary entry. It also regulates ciliary trafficking of hedgehog sig l transducer, Smoothened (22072986). Coordi tes the interactions between the BBSome and the IFT particle to regulate the removal of Ptch1 and Smo from cilia (25446516). Depletion leads to a Bardet-Biedl syndrome phenotype (22510444). We found no differences in cilia formation, cilia stability, or cilia length (in RPE1) LZTFL1 interagates with BBSome and regulates ciliary trafficking of BBSome and Smoothened (22072986) Viral treatment of HBECs resulted in significantly less amount of ciliated HBECs … in LZTFL1 shRNA‐infected cells (26364604)

Model organism evidence

Mus musculus (2 references)

Compared to wildtype (WT), LZKO CB-MSCs had elongated primary cilia with tapered tips and increased levels of peroxisome proliferator-activated receptor γ (PPARγ), a key transcription factor that favors adipogenesis, and nuclear glucocorticoid receptor (GR), a transcription factor involved in Pp

Bardet-Biedl syndrome (BBS) is an inherited ciliopathy affecting multiple organs and systems with wide clinical and genetic heterogeneity.

PMIDs: 39662832, 38801250