18. Muscle in health and disease - Robson Flashcards
What is the pattern of nuclei in a skeletal muscle cell?
Multinucleate cell
Where are the nuclei located in cells of skeletal muscle?
Nuclei are positioned peripherally
How long are skeletal muscle fibres?
10cm
What is the diameter of skeletal muscle fibres?
Vary between 10-100 micrometers
What is the significance of centrally located nuclei in skeletal muscle fibres?
Satellite cells have been activated to repair damaged muscle (nucleus moves back into the periphery once repaired)
What is the endomysium?
Aereolar connective tissue - covers each muscle fibre/muscle cell
What is the perimysium?
Connective tissue sheath - surrounds a bundle of muscle fibres from 10-100 - forms a muscle fascicle
What is the epimysium?
Fibroelastic tissue surrounding a muscle
How many muscle fibres are contained within a muscle fascicle?
Between 10-100
What is the centre of a skeletal muscle cell composed of?
Nuclei are peripheral so the centre is full of actin and myosin contractile proteins
Is skeletal muscle striated muscle? Why?
Yes - sometimes called striated muscle
This is because the actin and myosin form streaks - striated appearance
What is the shape of nuclei in the periphery of skeletal muscle cells?
Flattened
What is the shape of nuclei in the centre of skeletal muscle cells?
More rounded and bigger
What is skeletal muscle innervated by?
What does the innervation of the skeletal muscle lead to?
Innervated by motor neurones - initiate the contraction of the muscle
What chemical is involved in the innervation of skeletal muscle and where are these released from?
What are the receptors involved with this?
Ach
Ach released from the synapse terminal
Activation of nicotinic Ach receptor on the muscle fibre
Muscle contraction is initiated
How many muscle fibres are innervated by one motor neurone?
Between 5-200 muscle fibres
What is a motor unit?
These are the 5-200 muscle fibres that are all innervated by the same action potential from the same motor neurone - these fibres all contract at the same time
What is the significance of the loss of a single motor neurone?
Then all the muscle fibres that are innervated by this motor neurone will cease to function - i.e. the whole motor unit will cease to function
What is the effect on the shape of the skeletal muscle fibre if there is a loss of the innervating motor neurone?
Loss of innervating motor neurone
Muscle fibres are no longer innervated - become deinnervated and are no longer used
These muscle fibres undergo atrophy and become angulated in shape
What is the process of reinnervation?
Deinnervated muscle fibres - start to atrophy and releases stress signals to neighbouring muscle fibres (at the neuromuscular junction)
Causes neighbouring motor neurones to sprout extra ends to innervated these deinnervated muscle fibres
Where are stress signals from deinnervated muscle fibres sent to?
Neuromuscular junction of neighbouring muscle fibres - want to signal to the motor neurone innervated neighbouring motor unit
What is the main consequence of reinnervation?
More muscle fibres are being innervated by the same motor neurone - the size of the motor unit is increasing
Fibres are now more grouped together
Results in switch in the phenotype of the neighbouring muscle fibres and hence alters proper functioning of the muscle
What is the phenotype of a muscle fibre dependent on?
Dependent on the frequency of stimulation of the motor unit by the motor neurone
The frequency influences whether you have fast or slow twitch fibres
How does reinnervtion result in a switch in the phenotype of muscle fibres?
If fibres from slow frequency motor neurones die and are now innervated by fibres from high frequency motor neurones - these fibres will switch in the phenotype
What is the common name given to infantile hypotonia?
Floppy baby syndrome
What are the two different types of infantile hypotonia?
One affecting type 1 muscle fibres
One affecting type 2 muscle fibres
What type of metabolism is undergone by type 1 muscle fibres?
Mainly aerobic metabolism
What type of metabolism is undergone by type 2 muscle fibres?
Mix of aerobic and anaerobic metabolism
What are the two different types of infantile hypotonia and how do these present differently?
One - hypertrophy of the type 1 muscle fibres and disproportion between the size of the muscle fibres
Two - hypertrophy of the type 2 muscle fibres and disproportion between the size of the muscle fibres
The phenotype between these two is actually the same
Are type 1 muscle fibres slow or fast contracting?
Slow contracting/slow twitch
Are type 2 muscle fibres slow or fast contracting?
Fast contracting/fast twitch
How can you distinguish between the two types of infantile hypotonia?
Using an ATP scan and looking at the ATP levels being used for contraction of the fibre
Can a H&E scan be used to distinguish between the two types of infantile hypotonia?
No
When does infantile hypotonia arise?
Congenital
What is the effect of infantile hypotonia on the individual in later life?
Individual can undertake normal daily activity and live normal lives but cannot do such intense sports and they get tired a lot faster
Does the hypotonia in infantile hypotonia improve in later life?
Why?
Fibres start to improve as the infant gets older - there is a lot of continued growth of the muscle postnatally
Do type 1 muscle fibres appear pale or dark?
Pale
Do type 2 muscle fibres appear pale or dark?
Dark
What is sarcopenia?
The loss of skeletal muscle strength and mass as a result of ageing