Related Experiment Video
Updated: May 11, 2026
06:14
Modeling Human Cerebellar Development In Vitro in 2D Structure
Published on: September 16, 2022
α6 integrin subunit regulates cerebellar development.
Giovanni Marchetti1, Adèle De Arcangelis, Véronique Pfister
1IGBMC Institut de Génétique et de Biologie Moléculaire et Cellulaire, Department of Development and Stem Cells, CNRS UMR7104, Inserm U964, Université de Strasbourg, Illkirch, France. giovanni.marchetti@unice.fr
Cell Adhesion & Migration
|June 1, 2013
Summary
Alpha-6 integrin is crucial for cerebellum development. Its deletion in Bergmann glial cells disrupts fissure formation, impacting cerebellar foliation patterns.
Area of Science:
- Neuroscience
- Developmental Biology
- Cell Biology
Background:
- Basal lamina mutations disrupt cortical basement membrane, leading to disorganized cerebral and cerebellar cortices.
- Alpha-6 integrin (α6) is linked to cortical lamination defects and neural ectopias.
Purpose of the Study:
- To investigate the specific role of α6 integrin in the central nervous system (CNS).
- To generate and analyze α6 conditional knockout mice in neuronal and glia precursors using the Nestin-Cre line.
Main Methods:
- Generation of α6 conditional knockout mice (α6 fl/fl;nestin-Cre).
- Analysis of cerebral cortex development in mutant animals.
- Assessment of cerebellum foliation, granule cell (GC) proliferation and migration.
- Examination of Bergmann glial (BG) scaffold morphology, fiber outgrowth, and actin cytoskeleton.
Main Results:
- Cerebral cortex development was normal in α6 (fl/fl;nestin-Cre) mutants.
- The cerebellum exhibited foliation pattern defects.
- GC proliferation and migration remained unaffected.
- Abnormalities in BG fiber morphology, reduced outgrowth, and altered actin cytoskeleton were observed.
Conclusions:
- Alpha-6 integrin receptors are essential in Bergmann glial cells.
- α6 integrin is required for proper fissure formation during cerebellum morphogenesis.
- These findings highlight a specific role for α6 integrin in cerebellar development beyond cortical lamination.
More Related Videos
Related Concept Videos
01:10Integrins
Animal and protozoan cells do not have cell walls to help maintain shape and provide structural stability. Instead, these eukaryotic cells secrete a sticky mass of carbohydrates and proteins into the spaces between adjacent cells. This network of proteins and molecules is called an extracellular matrix or ECM.
Some ECM proteins assemble into a basement membrane to which the remaining components adhere. Proteoglycans typically form the bulk of the ECM while fibrous proteins, like collagen,...
Some ECM proteins assemble into a basement membrane to which the remaining components adhere. Proteoglycans typically form the bulk of the ECM while fibrous proteins, like collagen,...
01:17Intracellular Signaling Affects Focal Adhesions
Integrins act both as extracellular input receivers and as intracellular processing activators. As their name suggests, integrins are entirely integrated into the membrane structure. Their hydrophobic membrane-spanning regions interact with the phospholipid bilayer's hydrophobic region. These membrane receptors provide extracellular attachment sites for effectors like hormones and growth factors. They activate intracellular response cascades when their effectors are bound and active.
Some...
Some...
01:15Activation of Integrins
Integrins bind ligands and transmit information from outside the cell to inside or vice-versa through an "outside-in signaling" or "inside-out signaling."
In "outside-in signaling," external factors in the extracellular space bind to exposed ligand binding sites on integrins. This causes the inactive protein to undergo a conformational change to become active. Integrins are often clustered on the cell membrane. Repetitive and regularly spaced ligand binding events provide an effective stimulus.
In "outside-in signaling," external factors in the extracellular space bind to exposed ligand binding sites on integrins. This causes the inactive protein to undergo a conformational change to become active. Integrins are often clustered on the cell membrane. Repetitive and regularly spaced ligand binding events provide an effective stimulus.
01:17Cerebellum: Anatomical Regions
The cerebellum, also known as the "little brain," is located in the posterior cranial fossa, inferior to the tentorium cerebelli and dorsal to the brainstem. It plays a significant role in motor control, coordination, and proprioception.
Cerebellar Structure
Externally, the cerebellum features a highly convoluted surface with numerous folia (narrow ridges) separated by shallow sulci (grooves). The cerebellum is divided into two hemispheres by a thin median structure known as the vermis. The...
Cerebellar Structure
Externally, the cerebellum features a highly convoluted surface with numerous folia (narrow ridges) separated by shallow sulci (grooves). The cerebellum is divided into two hemispheres by a thin median structure known as the vermis. The...
01:03Anchoring Junctions
Anchoring junctions are multiprotein complexes that help cells connect to other cells and the extracellular matrix. Anchoring junctions are present on the lateral and basal surfaces of cells, providing strong and flexible connections. Focal adhesions are often formed due to cell interactions with the ECM substrata, which initiate signal transduction via kinase cascades and other mechanisms. Together, they provide stability and tissue integrity. There are three types of anchoring junctions:...
01:12Role Of Notch Signalling In Intestinal Stem Cell Renewal
Notch signaling was first discovered in Drosophila melanogaster, where it is involved in cell lineage differentiation. Notch signaling regulates the maintenance and differentiation of intestinal stem cells or ISCs by controlling the expression of atonal homolog 1 or Atoh1. Atoh1 directs cells to differentiate into secretory cells.
Direct cell-to-cell contact is needed for the activation of Notch signaling. The signal is initiated when a notch ligand binds to a receptor on an adjacent cell, also...
Direct cell-to-cell contact is needed for the activation of Notch signaling. The signal is initiated when a notch ligand binds to a receptor on an adjacent cell, also...