Add to ORKGBardet-Biedl syndrome (BBS), a ciliopathy, is a rare genetic condition characterised by retinal degeneration, obesity, kidney failure, and cognitive impairment. In spite of progress made in our general understanding of BBS aetiology, the molecular and cellular mechanisms underlying cognitive impairment in BBS remain elusive. Here, we report that the loss of BBS proteins causes synaptic dysfunction in principal neurons, providing a possible explanation for the cognitive impairment phenotype observed in BBS patients. Using synaptosomal proteomics and immunocytochemistry, we demonstrate the presence of Bbs proteins in the postsynaptic density (PSD) of hippocampal neurons. Loss of Bbs results in a significant reduction of dendritic spines in pr...
Primary cilia are key sensory organelles whose dysfunction leads to ciliopathy disorders such as Bar...
Primary cilia are evolutionarily conserved organelles required in a number of signalling pathways in...
SummaryPrimary cilium dysfunction affects the development and homeostasis of many organs in Bardet-B...
Bardet-Biedl syndrome (BBS), a ciliopathy, is a rare genetic condition characterised by retinal dege...
International audienceBardet-Biedl syndrome (BBS), a ciliopathy, is a rare genetic condition charact...
Bardet-Biedl syndrome (BBS) is a heterogeneous disorder characterized by complex symptoms, including...
Bardet-Biedl syndrome (BBS) is a genetically heterogeneous inherited human disorder displaying a ple...
Bardet-Biedl syndrome (BBS) is a genetically heterogeneous inherited human disorder displaying a ple...
Primary cilia regulate an expanding list of signaling pathways in many different cell types. It is l...
In the brain, primary cilia are found on most, if not all, central neurons. The importance of neuron...
Primary cilia are microtubule-based organelles present on most cells that regulate many physiologica...
<div><p>Primary cilia regulate an expanding list of signaling pathways in many different cell types....
<div><p>Bardet-Biedl Syndrome (BBS, MIM#209900) is a genetically heterogeneous disorder with pleiotr...
Bardet-Biedl Syndrome (BBS, MIM#209900) is a genetically heterogeneous disorder with pleiotropic phe...
SummaryPrimary cilium dysfunction underlies the pathogenesis of Bardet-Biedl syndrome (BBS), a genet...
Primary cilia are key sensory organelles whose dysfunction leads to ciliopathy disorders such as Bar...
Primary cilia are evolutionarily conserved organelles required in a number of signalling pathways in...
SummaryPrimary cilium dysfunction affects the development and homeostasis of many organs in Bardet-B...
Bardet-Biedl syndrome (BBS), a ciliopathy, is a rare genetic condition characterised by retinal dege...
International audienceBardet-Biedl syndrome (BBS), a ciliopathy, is a rare genetic condition charact...
Bardet-Biedl syndrome (BBS) is a heterogeneous disorder characterized by complex symptoms, including...
Bardet-Biedl syndrome (BBS) is a genetically heterogeneous inherited human disorder displaying a ple...
Bardet-Biedl syndrome (BBS) is a genetically heterogeneous inherited human disorder displaying a ple...
Primary cilia regulate an expanding list of signaling pathways in many different cell types. It is l...
In the brain, primary cilia are found on most, if not all, central neurons. The importance of neuron...
Primary cilia are microtubule-based organelles present on most cells that regulate many physiologica...
<div><p>Primary cilia regulate an expanding list of signaling pathways in many different cell types....
<div><p>Bardet-Biedl Syndrome (BBS, MIM#209900) is a genetically heterogeneous disorder with pleiotr...
Bardet-Biedl Syndrome (BBS, MIM#209900) is a genetically heterogeneous disorder with pleiotropic phe...
SummaryPrimary cilium dysfunction underlies the pathogenesis of Bardet-Biedl syndrome (BBS), a genet...
Primary cilia are key sensory organelles whose dysfunction leads to ciliopathy disorders such as Bar...
Primary cilia are evolutionarily conserved organelles required in a number of signalling pathways in...
SummaryPrimary cilium dysfunction affects the development and homeostasis of many organs in Bardet-B...