Synapse formation completes
the wiring of the nervous system

Synapse Formation in the Peripheral and Central Nervous System

Synapses: the basic computation units
in the brain
Human brain consists of 1011 neurons that form a network with 1014 connections
The number and specificity of synaptic connection needs to be precisely controlled
Changes of synaptic connections and synaptic strength are the basis of information processing and memory formation

Aberrant synaptic connectivity
and synaptic function lead to disease states
Loss of synapses in Alzheimer’s disease
In epilepsy excessive synapse formation and synaptic misfunction are observed
Genes associated with mental retardation and schizophrenia have synaptic functions
Paralysis after spinal cord injuries

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Central Synapses and
Neuromuscular Junctions (NMJs)
Neuron-neuron and neuron-muscle synapses develop by similar mechanisms
NMJs are larger, more accessible and simpler than central synapses therefore the molecular mechanisms of synapse formation are best understood for the NMJ

Structure of the neuromuscular junction
Mature NMJs consist of three cell types
Motor nerve
Muscle cell
Schwann cells
 All three cell types adopt a highly specialized organization that ensures proper synaptic function

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General Features of Synapse Formation
1) The pre- and post-synaptic cell organize each others organization (bi-directional signaling)
2) Synapses mature during development
 widening of synaptic cleft, basal lamina
 transition from multiple innervation to 1:1
3) Muscle and nerve contain components required for synaptogenesis (vesicles, transmitter, ACh-R)
 “reorganization”

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Clustering of ACh-R:
A) Aggregation of existing receptors

Clustering of ACh-R:
B) Local synthesis of receptors

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Agrin

Agrin signals through MuSK

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Summary of mutant phenotypes
Agrin -/-: few ACh-R clusters, overshooting of axons
MuSK -/-: no ACh-R clusters, overshooting of axons
Rapsyn -/-: no ACh-R clusters, but higher receptor levels in synaptic area, only limited overshooting
Pre-synaptic defects in all mutants, due to the lack of retrograde signals from the muscle

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Neuregulin (ARIA)
Acetylcholine receptor inducing activity
Expressed in motor neuron and in muscle
Binds and activates receptor tyrosine kinases on the muscle (erbB2, erbB3, erbB4)
Signals through MAP-kinase pathway
Leads to upregulation of ACh-R expression in sub-synaptic nuclei

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Clustering of ACh-R:
B) Local synthesis of receptors

Neural activity represses ACh-R synthesis in non-synaptic areas

Three neural signals for the induction of postsynaptic differentiation
Agrin: aggregation of receptors in the muscle membrane
Neuregulin: by upregulation of ACh-R expression in sub-synaptic nuclei
ACh/neural activity: downregulation of ACh-R expression in extra-synaptic nuclei

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Laminin 11 affects presynaptic differentiation

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Analogies of central synapses and NMJs
Overall structural similarities
Bi-directional signaling
Clustering of neurotransmitter receptors
Synaptic vesicles have similar components
 Synapse elimination during development

Differences between central synapses and NMJs
No basal lamina
No junctional folds but dendritic spines
Multiple innervation is common
Difference in neurotransmitters:
Excitatory synapses use glutamate
Inhibitory synapses use GABA (g-aminobutyric acid) and glycine
different neurotransmitter receptors

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Future directions/problems
Many factors that mediate synaptic differentiation in the CNS are not understood
Target specificity
Regeneration after injury is very low in CNS compared to PNS resulting in paralysis
Strategies to improve re-growth of axons and specific synapse formation