User profiles for M. R. Leroux
Michel LerouxSimon Fraser University Verified email at sfu.ca Cited by 15275 |
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 ciliopathies (…
of cilia causes diseases known as ciliopathies. The number of reported ciliopathies (…
The base of the cilium: roles for transition fibres and the transition zone in ciliary formation, maintenance and compartmentalization
Both the basal body and the microtubule‐based axoneme it nucleates have evolutionarily
conserved subdomains crucial for cilium biogenesis, function and maintenance. Here, we …
conserved subdomains crucial for cilium biogenesis, function and maintenance. Here, we …
[HTML][HTML] The sensory cilia of Caenorhabditis elegans.
The non-motile cilium, once believed to be a vestigial cellular structure, is now increasingly
associated with the ability of a wide variety of cells and organisms to sense their chemical …
associated with the ability of a wide variety of cells and organisms to sense their chemical …
[HTML][HTML] Comparative genomics identifies a flagellar and basal body proteome that includes the BBS5 human disease gene
Cilia and flagella are microtubule-based structures nucleated by modified centrioles termed
basal bodies. These biochemically complex organelles have more than 250 and 150 …
basal bodies. These biochemically complex organelles have more than 250 and 150 …
Basal body dysfunction is a likely cause of pleiotropic Bardet–Biedl syndrome
…, RC Johnsen, JC Cavender, R Alan Lewis, MR Leroux… - Nature, 2003 - nature.com
Bardet–Biedl syndrome (BBS) is a genetically heterogeneous disorder characterized primarily
by retinal dystrophy, obesity, polydactyly, renal malformations and learning disabilities. …
by retinal dystrophy, obesity, polydactyly, renal malformations and learning disabilities. …
The centrosomal protein nephrocystin-6 is mutated in Joubert syndrome and activates transcription factor ATF4
The molecular basis of nephronophthisis 1 , the most frequent genetic cause of renal failure
in children and young adults, and its association with retinal degeneration and cerebellar …
in children and young adults, and its association with retinal degeneration and cerebellar …
Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates
The evolutionarily conserved planar cell polarity (PCP) pathway (or noncanonical Wnt
pathway) drives several important cellular processes, including epithelial cell polarization, cell …
pathway) drives several important cellular processes, including epithelial cell polarization, cell …
MKS and NPHP modules cooperate to establish basal body/transition zone membrane associations and ciliary gate function during ciliogenesis
… Leroux; MKS and NPHP modules cooperate to establish basal body/transition zone membrane
associations and ciliary gate function during ciliogenesis. J Cell Biol 21 March 2011; 192 …
associations and ciliary gate function during ciliogenesis. J Cell Biol 21 March 2011; 192 …
Loss of C. elegans BBS-7 and BBS-8 protein function results in cilia defects and compromised intraflagellar transport
…, LM Quarmby, SR Wicks, MR Leroux - Genes & …, 2004 - genesdev.cshlp.org
Bardet-Biedl syndrome (BBS) is a genetically heterogeneous developmental disorder whose
molecular basis is largely unknown. Here, we show that mutations in the Caenorhabditis …
molecular basis is largely unknown. Here, we show that mutations in the Caenorhabditis …
Functional coordination of intraflagellar transport motors
Cilia have diverse roles in motility and sensory reception, and defects in cilia function
contribute to ciliary diseases such as Bardet–Biedl syndrome (BBS). Intraflagellar transport (IFT) …
contribute to ciliary diseases such as Bardet–Biedl syndrome (BBS). Intraflagellar transport (IFT) …