Cilia, ciliopathies and hedgehog-related forebrain developmental disorders

A Andreu-Cervera, M Catala… - Neurobiology of …, 2021 - Elsevier
Abstract Development of the forebrain critically depends on the Sonic Hedgehog (Shh)
signaling pathway, as illustrated in humans by the frequent perturbation of this pathway in …

GLI3: a mediator of genetic diseases, development and cancer

SJ Matissek, SF Elsawa - Cell Communication and Signaling, 2020 - Springer
The transcription factor GLI3 is a member of the Hedgehog (Hh/HH) signaling pathway that
can exist as a full length (Gli3-FL/GLI3-FL) or repressor (Gli3-R/GLI3-R) form. In response to …

Evolution and development of interhemispheric connections in the vertebrate forebrain

R Suárez, I Gobius, LJ Richards - Frontiers in human neuroscience, 2014 - frontiersin.org
Axonal connections between the left and right sides of the brain are crucial for bilateral
integration of lateralized sensory, motor, and associative functions. Throughout vertebrate …

The multifaceted roles of primary cilia in the development of the cerebral cortex

K Hasenpusch-Theil, T Theil - Frontiers in cell and developmental …, 2021 - frontiersin.org
The primary cilium, a microtubule based organelle protruding from the cell surface and
acting as an antenna in multiple signaling pathways, takes center stage in the formation of …

The multifaceted roles of Slits and Robos in cortical circuits: from proliferation to axon guidance and neurological diseases

H Blockus, A Chédotal - Current opinion in neurobiology, 2014 - Elsevier
Highlights•Slit–Robo guide thalamocortical projections from their birthplace to the cortex.•Slit–
Robo regulate radial as well as tangential migration of cortical neurons.•Slit–Robo affect …

Identification of ancestral gnathostome Gli3 enhancers with activity in mammals

S Ali, M Abrar, I Hussain, F Batool… - Development …, 2024 - Wiley Online Library
Abnormal expression of the transcriptional regulator and hedgehog (Hh) signaling pathway
effector Gli3 is known to trigger congenital disease, most frequently affecting the central …

Cilia and extracellular vesicles in brain development and disease

R Ma, L Chen, N Hu, S Caplan, G Hu - Biological Psychiatry, 2024 - Elsevier
Primary and motile cilia are thin, hair-like cellular projections from the cell surface involved
in movement, sensing, and communication between cells. Extracellular vesicles (EVs) are …

The role of primary cilia in corpus callosum formation is mediated by production of the Gli3 repressor

C Laclef, I Anselme, L Besse, M Catala… - Human molecular …, 2015 - academic.oup.com
Agenesis of the corpus callosum (AgCC) is a frequent brain disorder found in over 80
human congenital syndromes including ciliopathies. Here, we report a severe AgCC in …

The tumor suppressor Nf2 regulates corpus callosum development by inhibiting the transcriptional coactivator Yap

A Lavado, M Ware, J Paré, X Cao - Development, 2014 - journals.biologists.com
The corpus callosum connects cerebral hemispheres and is the largest axon tract in the
mammalian brain. Callosal malformations are among the most common congenital brain …

Cell-autonomous hedgehog signaling is not required for cyst formation in autosomal dominant polycystic kidney disease

M Ma, E Legué, X Tian, S Somlo… - Journal of the American …, 2019 - journals.lww.com
Background PKD1 or PKD2, the two main causal genes for autosomal dominant polycystic
kidney disease (ADPKD), encode the multipass transmembrane proteins polycystin-1 (PC1) …