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Genes and molecular pathways underpinning ciliopathies
Motile and non-motile (primary) cilia are nearly ubiquitous cellular organelles. The
dysfunction of cilia causes diseases known as ciliopathies. The number of reported …
dysfunction of cilia causes diseases known as ciliopathies. The number of reported …
Establishing and regulating the composition of cilia for signal transduction
The primary cilium is a hair-like surface-exposed organelle of the eukaryotic cell that
decodes a variety of signals—such as odorants, light and Hedgehog morphogens—by …
decodes a variety of signals—such as odorants, light and Hedgehog morphogens—by …
[HTML][HTML] Ciliopathies and the kidney: a review
Primary cilia are specialized sensory organelles that protrude from the apical surface of most
cell types. Over the past two decades, they have been found to play important roles in tissue …
cell types. Over the past two decades, they have been found to play important roles in tissue …
Open sesame: how transition fibers and the transition zone control ciliary composition
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 …
perform these functions, cilia must maintain a composition distinct from those of the …
BBSome trains remove activated GPCRs from cilia by enabling passage through the transition zone
A diffusion barrier at the transition zone enables the compartmentalization of signaling
molecules by cilia. The BBSome and the small guanosine triphosphatase Arl6, which …
molecules by cilia. The BBSome and the small guanosine triphosphatase Arl6, which …
[HTML][HTML] The ciliary transition zone: finding the pieces and assembling the gate
Eukaryotic cilia are organelles that project from the surface of cells to fulfill motility and
sensory functions. In vertebrates, the functions of both motile and immotile cilia are critical for …
sensory functions. In vertebrates, the functions of both motile and immotile cilia are critical for …
The molecular machines that traffic signaling receptors into and out of cilia
MV Nachury - Current opinion in cell biology, 2018 - Elsevier
Cilia are surface-exposed organelles that dynamically concentrate signaling molecules to
organize sensory, developmental and homeostatic pathways. Entry and exit of signaling …
organize sensory, developmental and homeostatic pathways. Entry and exit of signaling …
A CRISPR-based screen for Hedgehog signaling provides insights into ciliary function and ciliopathies
Primary cilia organize Hedgehog signaling and shape embryonic development, and their
dysregulation is the unifying cause of ciliopathies. We conducted a functional genomic …
dysregulation is the unifying cause of ciliopathies. We conducted a functional genomic …
How the ciliary membrane is organized inside-out to communicate outside-in
Cilia, organelles that move to execute functions like fertilization and signal to execute
functions like photoreception and embryonic patterning, are composed of a core of nine-fold …
functions like photoreception and embryonic patterning, are composed of a core of nine-fold …
The Joubert–Meckel–nephronophthisis spectrum of ciliopathies
The Joubert syndrome (JS), Meckel syndrome (MKS), and nephronophthisis (NPH)
ciliopathy spectrum could be the poster child for advances and challenges in Mendelian …
ciliopathy spectrum could be the poster child for advances and challenges in Mendelian …