Physiology and Pharmacology of the Skeletal Neuromuscular Junction Flashcards

Professor John Peters

1
Q

name the three types of muscle found in the body

A

cardiac
smooth
skeletal

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2
Q

what is skeletal muscle innervated by?

A

motor neurones

with myelinated axons and cell bodies in the spinal cord/brain stem

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3
Q

the neurone and the number of fibres that it innervates are know as what?

A

the motor unit

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4
Q

what chemical transmitter is found in skeletal muscle?

A

acetylcholine (ACh)

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5
Q

presynaptic terminal (bouton) of the alpha motor neurone synapses at what region of skeletal muscle fibres?

A

endplate

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6
Q

name the 4 key features of the skeletal neuromuscular junction

A
  1. terminal bouton with Schwann cells
  2. synaptic vesicles
  3. synaptic cleft
  4. end plate region
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7
Q

what are seen at active zones?

A

vesicles containing ACh

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8
Q

what receptor does ACh act on?

A

Nicotinic ACh receptor

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9
Q

what channels are concerned in he pre-synaptic process?

A

voltage-gated Ca2+ channels

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10
Q

what receptors does ACh activate?

A

nicotinic ACh receptors

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11
Q

when ACh is absent, is the gate open or closed?

A

closed

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12
Q

when does the receptor open?

A

when 2 molecules of ACh bond

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13
Q

Na+ efflux and K+ influx

true or false?

A

false

Na+ influx and K+ efflux

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14
Q

what is greater:

influx of Na+ or efflux of K+?

A

influx of Na+

net result is depolarisation known as end plate potential is generated

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15
Q

where is ACh stored and concentrated?

A

vesicles

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16
Q

what is one vesicle worth ACh known as?

17
Q

what is the electrical response to one quantum of transmitter known as?

A

miniature endplate potential

multiple m.e.p.p summates to the end plate potential

18
Q

is the e.p.p. is large enough, what does it trigger the opening of?

A

voltage-activated Na+ channels - all or nothing response

19
Q

where does the action potential enter in muscle?

A

transverse (T) tubules

releases Ca2+ which interacts with troponin causing contraction

20
Q

what are the symptoms of neuromyotonia/NMT/Isaac’s syndrome?

A

muscle cramps, stiffness and twitches

21
Q

what is the cause of neuromyotonia/NMT/Isaac’s syndrome?

A

autoimmune origin - antibodies against voltage gated K+ channels

22
Q

what is the drug treatment in neuromyotonia/NMT/Isaac’s syndrome?

A

carbamazepine and phenytoin

23
Q

what are the symptoms in Lambert-Eaton Myasthenic Syndrome (LEMS)?

A

muscle weakness - rare

24
Q

what is Lambert-Eaton Myasthenic Syndrome (LEMS)

associated with?

A

small cell carcinoma of the lung

25
what is the cause of Lambert-Eaton Myasthenic Syndrome (LEMS)?
autoimmune origin - antibodies against voltage gated Ca2+ channels so reduced release of ACh
26
what is the drug treatment in Lambert-Eaton Myasthenic Syndrome (LEMS)?
anticholinesterases - pyridostigmine | potassium channel blockers - 3,4-diaminopyridine
27
what are the symptoms of Myasthenia Gravis (MG)?
progressively increasing muscle weakness during activity - eye and eyelid muscle
28
what is the cause of Myasthenia Gravis (MG)?
autoimmune origin - antibodies against nicotinic ACh receptors
29
what is the drug treatment in Myasthenia Gravis (MG)?
anticholinesterases - edrophonium for diagnosis, pyridostigmine for long term treatment immunosuppressant agents - azathioprine
30
what is the botulinum toxin related to?
tetanus
31
what is the other name for botulinum toxin?
botox
32
what drugs Reduce the amplitude of the endplate potential (e.p.p.) to below the threshold for muscle fibre action potential generation?
curare-like compounds - competitive antagonists of nicotinic ACh receptors vecuronium and atracurium
33
alpha-motor neurone and all the skeletal muscle fibres that it innervates is the...
motor unit
34
The action of ACh is terminated its hydrolysis of what?
acetylcholinesterase (AChE)
35
Botulism is caused by botulinum toxic which causes...
failure of ACh release - paralysis