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Brain in Health & Disease > L1 - Neuromuscular Junction > Flashcards

L1 - Neuromuscular Junction Flashcards

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1
Q

What is ptosis?

A

Drooping of the upper or lower eyelid

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2
Q

What is a transmission disorder?

A

Faulty communication between nerves and muscles that leads to muscle weakness

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3
Q

What are 3 types of transmission disorder?

A

Axon fails to deliver strong signal
Fibre fails to respond to signal
Enhanced signal leads to uncontrolled activation

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4
Q

How does the neuromuscular junction work?

A
  1. action potential arrives to sarcoplasmic reticulum (SR)
  2. release of Ca
  3. muscle contracts
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5
Q

What three cells are involved in the NMJ?

A

Motor neuron
Schwann cell
Muscle cell

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6
Q

What neurotransmitter is present on the NMJ?

A

Acetylcholine

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7
Q

What is between the neuron and the muscle cell?

A

Basal lamina

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8
Q

What is the basal lamina?

A

Connective tissue crucial for maintain structure of NMJ and its function

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9
Q

What enzyme is in the basal lamina and what else is there?

A

Acetylcholinesterase associated with collagen

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10
Q

What structure is on the postsynaptic membrane and why?

A

Deep infolding of the sarcolemma (secondary synaptic folds) with anchored AChRs on the crests.
Accumulate as much receptor as possible and increase efficiency of transmission

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11
Q

What does acetylcholinesterase do?

A

Hydrolyses acetylcholine to transport it back into nerve terminal.
A serine in the active site does it

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12
Q

Why is acetylcholinesterase involved in disease?

A

Nerve gases and neurotxoins inhibit it by reacting with the same Serine - extending the period of membrane depolarisation

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13
Q

What are the two major events in development of NMJ?

A
  1. Receptors aggregating into clusters

2. Specialisation of nuclei sitting right underneath junction along muscle fibre

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14
Q

How many more AChRs are at the NMJ?

A

1000x more concentrated than at extra synaptic places

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15
Q

What experiment was used to show clustering of receptors?

A

Removed nerve and muscle fibre leaving only basal lamina

Signal sufficient for formation of synaptic folds rich in ACh

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16
Q

What fish was used to investigate thw NMJ and why?

A

Electric ray

Has 500 end places capable of releasing electric currents of 100V

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17
Q

What protein was eventually found to promote clustering?

A

Agrin

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18
Q

What are the features of agrin?

A

400 kDa proteoglycan
Secreted by motor neuron, muscle and schwann cell
Promotes clustering of AChR without enhancing AChR syn

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19
Q

What is the ratio of agrin to AChR at the NMJ and what does this show?

A

1:50 - 1:100

Clustering through signal rather than structural constraints

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20
Q

What was found to be the agrin receptor?

A

MuSK (muscle specific kinase)

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21
Q

How does agrin induce clustering?

A
  • Agrin interacts with MuSK, Masc complex
  • Musk is a receptor tyrosine kinase
  • MuSK activation leads to phosphorylation of rapsyn - a scaffolding protein and clusters AchRs
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22
Q

What is rapsyn?

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A

A scaffolding protein that clusters AChRs

23
Q

What is MASC and what is its function?

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A

myotube associated specificity component

Required to form agrin receptor

24
Q

What occurs in Lambert-Eaton syndrome?

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A

Autoantibodies to P/Q-type voltage gated calcium channels

25
What is hypothesised to be the origin of autoantibodies in Lambert-eaton?
Too much exposure to own protein | VGCCs known to be expressed by small cancer lung cells often present in this syndrome
26
What is the function impaired in Lambert-Eaton syndrome>
Downregulation of presynaptic Ca channels causes myasthenia by preventing NT release - not enough fibres firing
27
What occurs in Isaac's syndrome?
Autoantibodies to Voltage gated potassium channels
28
What are the internal symptoms of Isaac's syndrome
Increased NT release so persistent depolarisation enhanced muscle contraction
29
What are the external symptoms of Isaac's syndrome?
Painful cramps and spasms
30
What occurs in congenital myasthenic syndrome associated with episodic apnoea?
Mutations in CHAT impair catalytic efficiency of enzyme
31
What are the features and function of CHAT?
Located in terminals of cholinergic neurons | Catalyses reversible syn of acetylcholine from acetyl CoA and choline
32
Why causes acetylcholinesterase deficiency?
Mutations in collagen tail that anchors AChE
33
What are the internal symptoms of acetylcholinesterase deficiency?
Persistent presence of Ach in cleft causing repetitive binding to AChRs causing muscle fatigue and endplate myopathy
34
What do defects in repolarisation of the presynaptic membrane or elimination of ACh from cleft lead to?
Excessive transmission
35
What causes Seronegative myasthenia gravis?
Antibodies against MuSK
36
What causes congenital myasthenic syndrome?
Mutations in rapsyn
37
What are the internal symptoms of Myasthenia gravis and myasthenic syndrome?
Inhibition of agrin-MuSK signal destabilises NMJ
38
What causes postsynaptic congenital MG?
3 types of mutations in AChR genes
39
What are the 3 types of mutations in AChRs?
Slow channel syndrome Fast Channel Syndrome ACh deficiency
40
What is Slow channel syndrome?
channel takes longer to close and too much influx of NA takes place
41
What is Fast channel syndrome?
channel closes too quickly and not enough depolarisation
42
What is ACh deficiency?
Causes AChR to be unstable resulting in reduced receptors and deficient transmission
43
What are the external symptoms of Myasthenia gravis?
Strong in the morning but progressively weaker through day because of depletin of ACh
44
What is found in 80-90% of patients with MG?
Anti-AChR antibody
45
What causes neonatal MG?
Placental transfer of maternal AChR antibodies
46
What is arthrogryposis multiplex congenita?
MG associated in rare cases | Occurs when maternal AChR antibodies target fetal form
47
What does arthrogyrposis multiplex congenita cause and how?
Bent hands and feet due to excessive tendon force | Maternal antibodies had attacked fetal AchRs so contractinos crucial for development were not there
48
What does affecting the postsynaptic targets generally lead to?
Impaired transmsion and muscle weakness
49
What are the postsynaptic targets?
Rapsyn MuSK AchRs
50
What are the presynaptic and sypantic targets?
VGCC, VGKC, CHAT, ACHE
51
What are treatments of MG?
Cholinesterase inhibitors Thymectomy Medication to alter immune sys Immunoglobulin therapy
52
In congenital myasthenia gravis what is the treatment of a reduced response?
Cholinesterase inhibitors
53
In congenital myasthenia gravis what is the treatment of a increased response?
Open channel blockers of the AChR are used

Brain in Health & Disease (9 decks)

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