Neuromuscular junction
11 important questions on Neuromuscular junction
What is excitation-contracting coupling?
Which proteins are involved in fusion of ACh vesicles with the presynaptic membrane?
Synaptotagmin makes the connection tighter
Which proteins cluster the AChR?
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What is the effect of muscular diseases that result in too low EPP?
What is the quantal content?
--> n ACh quanta/pulse
What is the safety factor?
--> factor decreases with muscle diseases
What is the effect of disturbed NMJ?
Spontaneous activity
Cramps
What are causes of NMJ disorders?
mutation in encoding gene
intoxication
Name some examples of NMJ disorders
Lambert-Eaton myasthenic syndrome
Congenital myasthenic syndromes
Neuromyotonia
Miller Fisher syndrome
Inherited calcium channelopathies
What is the problem in Lambert-Eaton?
How can mouse models be used to analyze NMJ dysfunction?
- horizontal rods test = motor coordinatino/strenght
- whole-body plethysmography = measurement of respiration pattern
- grip strenght test = muscle strenght test
- inverted mesh hanging test = fatigable muscle strenght
- EMG = stimulation and recording of the muscle AP
- dissected muscle nerve preparations
- Ex vivo NMJ elecrophysiology
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