Cours: Neurodevelopmental Disorders
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Questions and Answers

What is the name of the human disorder caused by mutations in POMT1, POMGnT1, and Fukutin?

  • Reeler Syndrome
  • Spinal Muscular Atrophy
  • Cerebellar Hypoplasia
  • Cobblestone Lissencephaly (correct)
  • Which gene is mutated in Reeler mice, leading to abnormal cortical layering?

  • POMGnT1
  • Reelin (RELN) (correct)
  • Fukutin
  • POMT1
  • In the developing cerebellum, granule neuron precursors migrate in which direction?

  • Rostrally (correct)
  • Caudally
  • Dorsally
  • Ventrally
  • Which of the following cell types plays a role in granule cell migration towards the internal granule cell layer?

    <p>Bergmann glia (B)</p> Signup and view all the answers

    How does cerebellar development in mice compare to that in humans?

    <p>Cerebellar development in mice is significantly faster than in humans. (C)</p> Signup and view all the answers

    Which gene is specifically associated with periventricular heterotopia?

    <p>Arfgef2 (A)</p> Signup and view all the answers

    What abnormality is observed in the condition characterized by lissencephaly?

    <p>Double cortex (B)</p> Signup and view all the answers

    What type of cells are specifically referenced in the development context for SST+ cells?

    <p>Martinotti cells (A)</p> Signup and view all the answers

    What is the primary role of filamin A (FLNA) in relation to cortical development?

    <p>Actin-binding (B)</p> Signup and view all the answers

    Which of the following structures is affected during the migration of cortical interneurons?

    <p>Neocortex (D)</p> Signup and view all the answers

    What is the primary mode of migration for excitatory pyramidal neurons in the cerebral cortex during development?

    <p>Glial guided locomotion (B)</p> Signup and view all the answers

    Which neuronal precursor cells undergo radial migration in the cerebellum?

    <p>Purkinje precursor cells (C)</p> Signup and view all the answers

    During what phase of development does somal translocation occur for neuronal precursor cells?

    <p>Early development phase (B)</p> Signup and view all the answers

    What is the role of tangential migration in the cerebral cortex?

    <p>Immigration of inhibitory interneurons (A)</p> Signup and view all the answers

    Which layer of the cerebellum do the granule cells populate after migration?

    <p>Granular layer (C)</p> Signup and view all the answers

    What process allows neural crest cells to transition from an epithelial to a mesenchymal state?

    <p>Neural crest epithelial to mesenchymal transition (EMT) (D)</p> Signup and view all the answers

    Which of the following is NOT a derivative of neural crest cells?

    <p>Motor neurons (B)</p> Signup and view all the answers

    What is the primary function of radial glia cells during cortical development?

    <p>Self-renew and generate precursor cells (B)</p> Signup and view all the answers

    Which expression is used as a stop signal for migrating precursors?

    <p>Reelin (B)</p> Signup and view all the answers

    During the development of the cerebral cortex, where do precursor cells primarily originate?

    <p>Ventricular Zone (VZ) (C)</p> Signup and view all the answers

    What key factor influences the formation of neurons versus glial cells during nervous system development?

    <p>Cellular time of division (D)</p> Signup and view all the answers

    What type of cells migrate long distances to their final destination within the central nervous system?

    <p>Precursor cells (B)</p> Signup and view all the answers

    What role do glia play in the locomotion of neurons during development?

    <p>Provide pathways through glia-guided locomotion (D)</p> Signup and view all the answers

    What is a function of the basal radial glia cells in the outer subventricular zone?

    <p>They allow for increased neuronal cell number. (B)</p> Signup and view all the answers

    Which aspect of corticogenesis has shown an increase over evolutionary time in primates compared to rodents?

    <p>Timing and cell-type complexity. (D)</p> Signup and view all the answers

    Where do GABAergic interneurons originate?

    <p>Medial, caudal, or lateral ganglionic eminence. (C)</p> Signup and view all the answers

    What do interneurons use to form connections with excitatory neurons in the neocortex?

    <p>Synapses. (A)</p> Signup and view all the answers

    What is the significance of thalamocortical pathways in cortical areas?

    <p>They target specific areas in the cortex. (C)</p> Signup and view all the answers

    In the context of neuronal migration, what is the first step that occurs?

    <p>Ventricle-directed migration. (B)</p> Signup and view all the answers

    What is a primary role of the guidance cues in the telencephalon?

    <p>To direct interneuron migration. (B)</p> Signup and view all the answers

    What principle of corticogenesis is conserved between rodents and primates?

    <p>The basic principles themselves. (B)</p> Signup and view all the answers

    Study Notes

    Cell Migration in the Developing Nervous System

    • Cell migration is a crucial part of nervous system development.
    • Cell migration in the developing nervous system involves different stages, including proliferation, differentiation, connectivity, maturation, and pruning.
    • Aberrant development can lead to diseases.
    • Neural crest cells contribute to the peripheral nervous system (PNS).
    • Neural crest cells undergo specification, EMT/delamination, and migration.
    • Neural crest cells give rise to cranial (bone, cartilage, connective tissue), vagal (enteric neurons, sensory neurons, glial cells, smooth muscle, cardiac tissue), and trunk (sensory neurons, autonomic neurons, chromaffin cells, glial cells, melanocytes) derivatives.
    • Precursor cells in the central nervous system (CNS) migrate long distances to reach their final destinations.
    • The development of the cerebral cortex involves proliferation and migration.
    • Examples of proliferation stages are observed in E 8.5 in the ventricular zone (VZ).
    • Migration is evident from the VZ to the cortical layers.
    • Development of cortical and glial cells involves specific times and sequences.
    • Radial glia cells are stem cells of the developing cortex.
    • Radial glia undergo symmetric and asymmetric divisions, leading to gliogenesis.
    • Radial glia cells guide neuronal migration.
    • Cajal Retzius cells release Reelin as a stop signal for migrating precursors.
    • Apical progenitors/radial glia cells divide asymmetrically to self-renew and generate precursor cells.
    • Cortical plate development occurs from the inside out.
    • Birthdating with [H]-Thymidine can be used to identify the time of origin of neurons.
    • The basic arrangement of cortical areas in the brain is preserved across species.
    • Interneurons form synapses with different parts of excitatory neurons.
    • The diversity of GABAergic interneurons in the neocortex is essential for neural circuit function.
    • Interneurons originate in the medial, caudal, or lateral ganglionic eminence.
    • Guidance cues are essential for neuronal migration.
    • Ventricle-directed migration precedes layer-specific targeting in interneurons.
    • Milestones in cortical interneuron development include neurogenesis, tangential migration, laminar allocation, wiring, programmed cell death, and circuit refinement.
    • Cortical migration defects can be categorized as initiation, ongoing migration, lamination, and stop signal defects.
    • Reeler mice show aberrant layering of the cortex.
    • Cobblestone lissencephaly is a human disorder associated with cortical migration defects.
    • Cerebellar development involves granule neuron precursor migration and precerebellar nuclei precursors migration.
    • Granule cells send out parallel fibers before migrating along Bergmann glia fibers.
    • Cerebellar development is completed about three weeks after birth in mice.
    • Glutamatergic and GABAergic neurons have different origins.
    • Molecular connectivity in the cerebellum involves Purkinje cells, granule cells, and neurons.

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    Test your knowledge on neurodevelopmental disorders and their underlying genetic causes. This quiz covers key concepts such as gene mutations, neuronal migration, and structural brain abnormalities. Perfect for students studying neuroscience or related fields.

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