Signaling through the primary cilium

G Wheway, L Nazlamova, JT Hancock - Frontiers in cell and …, 2018 - frontiersin.org
The presence of single, non-motile “primary” cilia on the surface of epithelial cells has been
well described since the 1960s. However, for decades these organelles were believed to be …

Open sesame: how transition fibers and the transition zone control ciliary composition

FR Garcia-Gonzalo, JF Reiter - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Cilia are plasma membrane protrusions that act as cellular propellers or antennae. To
perform these functions, cilia must maintain a composition distinct from those of the …

The regulation of cilium assembly and disassembly in development and disease

L Wang, BD Dynlacht - Development, 2018 - journals.biologists.com
The primary cilium is an antenna-like organelle assembled on most types of quiescent and
differentiated mammalian cells. This immotile structure is essential for interpreting …

Primary cilia, ciliogenesis and the actin cytoskeleton: a little less resorption, a little more actin please

CEL Smith, AVR Lake, CA Johnson - Frontiers in cell and …, 2020 - frontiersin.org
Primary cilia are microtubule-based organelles that extend from the apical surface of most
mammalian cells, forming when the basal body (derived from the mother centriole) docks at …

Actin remodelling factors control ciliogenesis by regulating YAP/TAZ activity and vesicle trafficking

J Kim, H Jo, H Hong, MH Kim, JM Kim, JK Lee… - Nature …, 2015 - nature.com
Primary cilia exert a profound impact on cell signalling and cell cycle progression. Recently,
actin cytoskeleton destabilization has been recognized as a dominant inducer of …

Many genes—one disease? Genetics of Nephronophthisis (NPHP) and NPHP-associated disorders

S Srivastava, E Molinari, S Raman, JA Sayer - Frontiers in pediatrics, 2018 - frontiersin.org
Nephronophthisis (NPHP) is a renal ciliopathy and an autosomal recessive cause of cystic
kidney disease, renal fibrosis, and end-stage renal failure, affecting children and young …

Bardet–Biedl syndrome—Multiple kaleidoscope images: insight into mechanisms of genotype–phenotype correlations

L Florea, L Caba, EV Gorduza - Genes, 2021 - mdpi.com
Bardet–Biedl Syndrome is a rare non-motile primary ciliopathy with multisystem involvement
and autosomal recessive inheritance. The clinical picture is extremely polymorphic. The …

Genetics and mechanisms leading to human cortical malformations

DM Romero, N Bahi-Buisson, F Francis - Seminars in cell & developmental …, 2018 - Elsevier
Cerebral cortical development involves a complex series of highly regulated steps to
generate the laminated structure of the adult neocortex. Neuronal migration is a key part of …

Fetal lung underdevelopment is rescued by administration of amniotic fluid stem cell extracellular vesicles in rodents

L Antounians, VD Catania, L Montalva, BD Liu… - Science Translational …, 2021 - science.org
Fetal lung underdevelopment, also known as pulmonary hypoplasia, is characterized by
decreased lung growth and maturation. The most common birth defect found in babies with …

The ciliary membrane‐associated proteome reveals actin‐binding proteins as key components of cilia

P Kohli, M Höhne, C Jüngst, S Bertsch, LK Ebert… - EMBO …, 2017 - embopress.org
Primary cilia are sensory, antennae‐like organelles present on the surface of many cell
types. They have been involved in a variety of diseases collectively termed ciliopathies. As …