MSK - NMJ Flashcards

(54 cards)

1
Q

what innervates skeletal muscle?

A

fast conducting alpha motor neurons with myelinated axons and cell Bodies in the spinal cord or brain stem

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2
Q

where does the axon divide into unmyelinated branches?

A

near the muscle to innervate individual muscle fibres

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3
Q

what is the motor unit?

A

the neurone and the number of fibres it innervates

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4
Q

what is the end of the branches?

A

terminal bouton that synapses with the muscle membrane at NMJ

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5
Q

what does an action potential cause?

A

release of transmitter Ach

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6
Q

where exactly is the synapse?

A

endplate region of skeletal muscle fibre

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7
Q

what contains the Ach?

A

synaptic vesicles which cluster at active zones

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8
Q

where are the nicotinic Ach receptors?

A

junctional folds that face the active zones

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9
Q

where is Ach synthesised?

A

cytoplasm of bouton

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10
Q

where is Ach stored?

A

synaptic vesicles

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11
Q

how is Ach released from the vesicles into the clefts?

A

Ca2+ dependant release by exocytosis

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12
Q

what happens once Ach is released?

A

brief activation of nicotinic receptors by reversible binding

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13
Q

what terminated the binding of Ach?

A

acetylcholinesterase

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14
Q

what terminates the binding of Ach?

A

acetylcholinesterase

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15
Q

pre-synpatically, how is choline transported into the terminal?

A

choline transporter (symport with Na+)

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16
Q

what is combined to produce Ach and by which enzyme?

A

choline and acetyl coenzyme A

choline acetlytransferase

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17
Q

what concentrates Ach in the vesicles?

A

vesicular Ach transporter

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18
Q

what does arrival of the action potential at the terminal cause?

A

depolarisation
opening of voltage gated Ca2+ channels
Ca2+ entry to the terminal

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19
Q

what does Ca+ cause the docked vesicles to do?

A

undergo exocytosis

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20
Q

how many ACh molecules activate each nicotinic receptor?

A

2

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21
Q

what are nAChRs?

A

pentamers of glycoprotein subunits surrounding a central cation selective pore

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22
Q

when does the pore/gate open?

A

when ACh binds

23
Q

what is the channel equally permeable to and not permeable to?

A

Na+ and K+

anions

24
Q

what happens when the gate is open?

A

Na+ enters and K+ exits simultaneously

25
how is a depolarising end plate potential generated?
influx of Na+ is greater (driving force at resting potential) simultaneous opening of many nAChrs
26
what does each vesicle of Ach contain?
a quantum of neurotransmitter
27
what is a miniature end plate potential?
the electrical response to one quantum of transmitter
28
what is the graded electronic response?
when many MEPPs summate to produce an end plate potential
29
what is the condition for the end plate potential to cause contraction?
all or none reaction | it must exceed threshold to trigger contraction but this always occurs normally
30
what causes the contraction once the action potential is released?
release of Ca2+ from intracellular stores
31
how does the AP enter the muscle fibre?
transverse T tubules
32
what are transverse T tubules?
invaginations of cell membrane that dip into muscle cell
33
what do APs arriving at T tubules trigger?
release of Ca2+ from the SR
34
how does Ca2+ release cause the contraction?
by interacting with troponin assoc with myofibrils
35
what causes rapid termination of transmission?
hydrolysis of ACh by acetylcholinesterase
36
where do the end products choline and acetate go?
choline is taken up by the choline transporter | acetate diffuses from the synaptic cleft
37
how is the termination so rapid?
extremely efficient | some Ach molecules are hydrolysed before the transmitter has even bound to nicotinic receptors
38
which drugs reversibly block the action of AChE?
anti-cholinesterase
39
what is neuromyotonia?
disorders of muscle function including cramps, stiffness, slow relaxation and twitches
40
what causes acquired neuromyotonia?
AI | antibodies against K+ channels disrupt function
41
what do the antibodies in neuromyotonia cause?
hyper-excitability and repetitive firing | prolonged epp and repetitive AP discharge
42
what are used to treat neuromytonias?
anticonvulsants which block Na+ channels
43
main symptom of Lambert eaton myasthenic syndrome?
muscle weakness in limbs
44
what is assoc with LEMS?
small cell carcinoma of lung
45
origin of LEMS?
AI | antibodies against Ca+ channels
46
what are used to treat LEMS?
anticholinesterases to increase duration of ACh in cleft | potassium blockers to increase release of ACh
47
main symptoms of MG?
progressively increasing muscle weakness during period of activity eye and eyelid weakness
48
origin of MG?
antibodies against ACh receptors
49
treatment of MG?
anticholinestrases and immunosuppressants
50
what is botulin toxin?
endotoxin that acts at motor neurone terminals to irreversibly inhibit ACh release high death rate
51
what can a low dose of botulin toxin treat?
``` overactive muscles (dystopias) extra ocular muscles for squints or eyelids smoothing out wrinkles ```
52
what are curate like compounds?
interfere with the postsynaptic action of ACh by acting as competitive antagonists of the nicotinic receptors
53
what do curate like compounds cause?
reduction of amplitude of the epp to below the threshold for AP generation
54
what are curate like compounds used for?
induce reversible muscle paralysis in certain surgeries