Mapping the molecular and cellular complexity of cortical malformations

E Klingler, F Francis, D Jabaudon, S Cappello - Science, 2021 - science.org
INTRODUCTION The cerebral cortex, or neocortex, is critical to key mammalian skills such
as language, sociability, and sensorimotor control. This structure consists of dozens of …

Genetics and mechanisms leading to human cortical malformations

DM Romero, N Bahi-Buisson, F Francis - Seminars in cell & developmental …, 2018 - Elsevier
Cerebral cortical development involves a complex series of highly regulated steps to
generate the laminated structure of the adult neocortex. Neuronal migration is a key part of …

[HTML][HTML] Dystroglycanopathy: from elucidation of molecular and pathological mechanisms to development of treatment methods

M Kanagawa - International Journal of Molecular Sciences, 2021 - mdpi.com
Dystroglycanopathy is a collective term referring to muscular dystrophies with abnormal
glycosylation of dystroglycan. At least 18 causative genes of dystroglycanopathy have been …

[HTML][HTML] Radial glia regulate vascular patterning around the developing spinal cord

RL Matsuoka, M Marass, A Avdesh, CSM Helker… - elife, 2016 - elifesciences.org
Vascular networks surrounding individual organs are important for their development,
maintenance, and function; however, how these networks are assembled remains poorly …

The roles of dystroglycan in the nervous system: insights from animal models of muscular dystrophy

AR Nickolls, CG Bönnemann - Disease Models & …, 2018 - journals.biologists.com
Dystroglycan is a cell membrane protein that binds to the extracellular matrix in a variety of
mammalian tissues. The α-subunit of dystroglycan (αDG) is heavily glycosylated, including a …

Protein O-mannosylation: one sugar, several pathways, many functions

M Koff, P Monagas-Valentin, B Novikov, I Chandel… - …, 2023 - academic.oup.com
Recent research has unveiled numerous important functions of protein glycosylation in
development, homeostasis, and diseases. A type of glycosylation taking the center stage is …

High degree of conservation of the enzymes synthesizing the laminin-binding glycoepitope of α-dystroglycan

MG Bigotti, A Brancaccio - Open biology, 2021 - royalsocietypublishing.org
The dystroglycan (DG) complex plays a pivotal role for the stabilization of muscles in
Metazoa. It is formed by two subunits, extracellular α-DG and transmembrane β-DG …

Dystroglycan maintains inner limiting membrane integrity to coordinate retinal development

R Clements, R Turk, KP Campbell… - Journal of …, 2017 - Soc Neuroscience
Proper neural circuit formation requires the precise regulation of neuronal migration, axon
guidance, and dendritic arborization. Mutations affecting the function of the transmembrane …

[HTML][HTML] Semaphorin 6A–Plexin A2/A4 Interactions with Radial Glia Regulate Migration Termination of Superficial Layer Cortical Neurons

Y Hatanaka, T Kawasaki, T Abe, G Shioi, T Kohno… - IScience, 2019 - cell.com
Precise regulation of neuronal migration termination is crucial for the establishment of brain
cytoarchitectures. However, little is known about how neurons terminate migration. Here we …

[HTML][HTML] Netrin-1 confines rhombic lip-derived neurons to the CNS

AR Yung, NR Druckenbrod, JF Cloutier, Z Wu… - Cell reports, 2018 - cell.com
During brainstem development, newborn neurons originating from the rhombic lip embark
on exceptionally long migrations to generate nuclei important for audition, movement, and …