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 …

Centrosome biogenesis and function: centrosomics brings new understanding

M Bettencourt-Dias, DM Glover - Nature reviews Molecular cell biology, 2007 - nature.com
Centrosomes, which were first described in the late 19th century, are found in most animal
cells and undergo duplication once every cell cycle so that their number remains stable, like …

A dynamic protein interaction landscape of the human centrosome-cilium interface

GD Gupta, É Coyaud, J Gonçalves, BA Mojarad, Y Liu… - Cell, 2015 - cell.com
The centrosome is the primary microtubule organizing center of the cells and templates the
formation of cilia, thereby operating at a nexus of critical cellular functions. Here, we use …

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 …

The centrosome is an actin-organizing centre

F Farina, J Gaillard, C Guérin, Y Couté… - Nature cell …, 2016 - nature.com
Microtubules and actin filaments are the two main cytoskeleton networks supporting
intracellular architecture and cell polarity. The centrosome nucleates and anchors …

53BP1 and USP28 mediate p53 activation and G1 arrest after centrosome loss or extended mitotic duration

F Meitinger, JV Anzola, M Kaulich, A Richardson… - Journal of Cell …, 2016 - rupress.org
In normal human cells, centrosome loss induced by centrinone—a specific centrosome
duplication inhibitor—leads to irreversible, p53-dependent G1 arrest by an unknown …

Basal body dysfunction is a likely cause of pleiotropic Bardet–Biedl syndrome

SJ Ansley, JL Badano, OE Blacque, J Hill, BE Hoskins… - Nature, 2003 - nature.com
Bardet–Biedl syndrome (BBS) is a genetically heterogeneous disorder characterized
primarily by retinal dystrophy, obesity, polydactyly, renal malformations and learning …

Cep164 mediates vesicular docking to the mother centriole during early steps of ciliogenesis

KN Schmidt, S Kuhns, A Neuner, B Hub… - Journal of Cell …, 2012 - rupress.org
Cilia formation is a multi-step process that starts with the docking of a vesicle at the distal
part of the mother centriole. This step marks the conversion of the mother centriole into the …

Assembly of centrosomal proteins and microtubule organization depends on PCM-1

A Dammermann, A Merdes - The Journal of cell biology, 2002 - rupress.org
The protein PCM-1 localizes to cytoplasmic granules known as “centriolar satellites” that are
partly enriched around the centrosome. We inhibited PCM-1 function using a variety of …

Centrosome composition and microtubule anchoring mechanisms

M Bornens - Current opinion in cell biology, 2002 - Elsevier
Centrosomes of animal cells and spindle pole bodies of fungi are the major microtubule
nucleating centers. Recent studies indicate that their capacity to organize microtubule arrays …