Genes and molecular pathways underpinning ciliopathies

JF Reiter, MR Leroux - Nature reviews Molecular cell biology, 2017 - nature.com
Motile and non-motile (primary) cilia are nearly ubiquitous cellular organelles. The
dysfunction of cilia causes diseases known as ciliopathies. The number of reported …

Cilia and mucociliary clearance

XM Bustamante-Marin… - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Mucociliary clearance (MCC) is the primary innate defense mechanism of the lung. The
functional components are the protective mucous layer, the airway surface liquid layer, and …

Ciliogenesis: building the cell's antenna

H Ishikawa, WF Marshall - Nature reviews Molecular cell biology, 2011 - nature.com
The cilium is a complex organelle, the assembly of which requires the coordination of motor-
driven intraflagellar transport (IFT), membrane trafficking and selective import of cilium …

The Chlamydomonas Genome Reveals the Evolution of Key Animal and Plant Functions

SS Merchant, SE Prochnik, O Vallon, EH Harris… - Science, 2007 - science.org
Chlamydomonas reinhardtii is a unicellular green alga whose lineage diverged from land
plants over 1 billion years ago. It is a model system for studying chloroplast-based …

A core complex of BBS proteins cooperates with the GTPase Rab8 to promote ciliary membrane biogenesis

MV Nachury, AV Loktev, Q Zhang, CJ Westlake… - Cell, 2007 - cell.com
Primary cilium dysfunction underlies the pathogenesis of Bardet-Biedl syndrome (BBS), a
genetic disorder whose symptoms include obesity, retinal degeneration, and nephropathy …

When cilia go bad: cilia defects and ciliopathies

M Fliegauf, T Benzing, H Omran - Nature reviews Molecular cell biology, 2007 - nature.com
Defects in the function of cellular organelles such as peroxisomes, lysosomes and
mitochondria are well-known causes of human diseases. Recently, another organelle has …

Functional architecture of olfactory ionotropic glutamate receptors

L Abuin, B Bargeton, MH Ulbrich, EY Isacoff… - Neuron, 2011 - cell.com
Ionotropic glutamate receptors (iGluRs) are ligand-gated ion channels that mediate
chemical communication between neurons at synapses. A variant iGluR subfamily, the …

IFT-A structure reveals carriages for membrane protein transport into cilia

SJ Hesketh, AG Mukhopadhyay, D Nakamura… - Cell, 2022 - cell.com
Intraflagellar transport (IFT) trains are massive molecular machines that traffic proteins
between cilia and the cell body. Each IFT train is a dynamic polymer of two large complexes …

The ciliopathies: an emerging class of human genetic disorders

JL Badano, N Mitsuma, PL Beales… - Annu. Rev. Genomics …, 2006 - annualreviews.org
Cilia and flagella are ancient, evolutionarily conserved organelles that project from cell
surfaces to perform diverse biological roles, including whole-cell locomotion; movement of …

Atypical Membrane Topology and Heteromeric Function of Drosophila Odorant Receptors In Vivo

R Benton, S Sachse, SW Michnick, LB Vosshall - PLoS biology, 2006 - journals.plos.org
Drosophila olfactory sensory neurons (OSNs) each express two odorant receptors (ORs): a
divergent member of the OR family and the highly conserved, broadly expressed receptor …