Primary cilia as dynamic and diverse signalling hubs in development and disease

P Mill, ST Christensen, LB Pedersen - Nature Reviews Genetics, 2023 - nature.com
Primary cilia, antenna-like sensory organelles protruding from the surface of most vertebrate
cell types, are essential for regulating signalling pathways during development and adult …

Mucociliary respiratory epithelium integrity in molecular defense and susceptibility to pulmonary viral infections

Adivitiya, MS Kaushik, S Chakraborty, S Veleri… - Biology, 2021 - mdpi.com
Simple Summary Mucociliary clearance constitutes an innate lung defense mechanism that
is primarily driven by ciliated cells. Respiratory mucus traps pathogens entering the airways …

Developmental toxicity of the novel PFOS alternative OBS in developing zebrafish: An emphasis on cilia disruption

J Huang, L Sun, JA Mennigen, Y Liu, S Liu… - Journal of hazardous …, 2021 - Elsevier
In recent years, sodium p-perfluorous nonenoxybenzene sulfonate (OBS) has emerged as a
substitute for PFOS with large demand and application in the Chinese market. However, little …

PPARγ interaction with UBR5/ATMIN promotes DNA repair to maintain endothelial homeostasis

CG Li, C Mahon, NM Sweeney, E Verschueren… - Cell reports, 2019 - cell.com
Using proteomic approaches, we uncovered a DNA damage response (DDR) function for
peroxisome proliferator activated receptor γ (PPARγ) through its interaction with the DNA …

Ciliary exclusion of Polycystin-2 promotes kidney cystogenesis in an autosomal dominant polycystic kidney disease model

RV Walker, JL Keynton, DT Grimes… - Nature …, 2019 - nature.com
The human PKD2 locus encodes Polycystin-2 (PC2), a TRPP channel that localises to
several distinct cellular compartments, including the cilium. PKD2 mutations cause …

Microtubule motors drive hedgehog signaling in primary cilia

M He, S Agbu, KV Anderson - Trends in cell biology, 2017 - cell.com
The mammalian Hedgehog (Hh) signaling pathway is required for development and for
maintenance of adult stem cells, and overactivation of the pathway can cause …

KIF13B establishes a CAV1-enriched microdomain at the ciliary transition zone to promote Sonic hedgehog signalling

KB Schou, JB Mogensen, SK Morthorst… - Nature …, 2017 - nature.com
Ciliary membrane composition is controlled by transition zone (TZ) proteins such as
RPGRIP1, RPGRIPL and NPHP4, which are vital for balanced coordination of diverse …

Multivalency regulates activity in an intrinsically disordered transcription factor

S Clark, JB Myers, A King, R Fiala, J Novacek… - Elife, 2018 - elifesciences.org
The transcription factor ASCIZ (ATMIN, ZNF822) has an unusually high number of
recognition motifs for the product of its main target gene, the hub protein LC8 (DYNLL1) …

[HTML][HTML] The genetics of autosomal recessive polycystic kidney disease (ARPKD)

P Goggolidou, T Richards - Biochimica et Biophysica Acta (BBA)-Molecular …, 2022 - Elsevier
ARPKD is a genetically inherited kidney disease that manifests by bilateral enlargement of
cystic kidneys and liver fibrosis. It shows a range of severity, with 30% of individuals dying …

[HTML][HTML] Dynein and intraflagellar transport

Y Hou, GB Witman - Experimental cell research, 2015 - ncbi.nlm.nih.gov
Intraflagellar transport (IFT) is the bi-directional movement of particles along the length of
cilia/flagella (terms used interchangeably here)(reviewed in Rosenbaum and Witman, 2002; …