Neuromuscular Junction: Development

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Main topics for this lecture • The neurobiological context.. • Motor Neurone birth • Neuromuscular synapse formation • Motor Neurone death • Neuromuscular synapse elimination • Molecular changes in ACh Receptors • Neonatal disease of motor units: Spinal Muscular Atrophy (SMA) • Development of myelin sheaths • Motor nerve sprouting and regeneration: recapitulation of development
The neurobiological context….
Page 1: Development of neuromuscular innerv
Page 5 and 6: Remodelling of connections is a fea
Page 7 and 8: Activity-dependent remodelling may
Page 9 and 10: Overview of motor units and their d
Page 11 and 12: Motor neurone cell bodies occupy th
Page 13 and 14: Mature NMJ’s are mononeuronally i
Page 15 and 16: Four cell types at the NMJ tSC KC N
Page 17 and 18: Neonatal muscle fibres are polyneur
Page 19 and 20: Summary of key stages in the develo
Page 21 and 22: Quantal Content (variance method) a
Page 24 and 25: The neural tube contains gradients
Page 26 and 27: Selective outgrowth of axons…
Page 28 and 29: http://www.bio.miami.edu/ktosney/
Page 30 and 31: Growth cones are semi-autonomous Re
Page 32 and 33: In immature spinal cord in vivo, ne
Page 34 and 35: Topographic projections are determi
Page 36 and 37: Myogenesis Myoblasts Myotubes Muscl
Page 39 and 40: Two types of synapse formation: FaS
Page 41 and 42: ACh Receptor maturation…
Page 43 and 44: Agrin clusters ACh receptors via Mu
Page 45 and 46: Neonate: AChR - γ Adult AChR - ε
Page 47 and 48: AChR-ε knockout mice continue to m
Page 49 and 50: Natural Motor Neurone Death…
Page 51 and 52: Target size regulates the number of
Page 53 and 54:
Muscle paralysis inhibits embryonic
Page 55 and 56:
Natural Synapse Elimination…
Page 57:
Physiological methods of measuring
Page 61 and 62:
Time lapse imaging of synapse elimi
Page 63 and 64:
Loss of motor neurones? Or eliminat
Page 65 and 66:
Motor unit size decreases postnatal
Page 67 and 68:
Neurones retract some of their syna
Page 69 and 70:
Neuronal/synaptic competition: “T
Page 71 and 72:
The rate of synapse elimination is
Page 73 and 74:
Transgenic expression of a growth f
Page 75 and 76:
We can determine motor unit size in
Page 77 and 78:
Motor unit sizes 2 days after parti
Page 79:
Motor units may compete in a “dom
Page 82 and 83:
Impaired NMJ form and function in n
Page 84 and 85:
Myelin formation…
Page 86 and 87:
Myelin forms from compacted Schwann
Page 88 and 89:
Regeneration…
Page 90 and 91:
MPN LPN SN 4DL
Page 92 and 93:
Axonal sprouting is preceded by Sch
Page 94 and 95:
Polyneuronal innervation (π) in re
Page 96 and 97:
…but some polyinnervation persist
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Neuromuscular Junction: Development

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