What surrounds bundles of muscle fibres, entire muscle and individual muscle fibres?
Give two features of red fibres (type I, slow twitch)?
2. Increased myoglobin
Give two features of white fibres (type II fast twitch)?
2. Large motor end-plates
Why are slow twitch fibres resistant to fatigue?
Greater ability to regenerate ATP
In type I muscle fibres - what colour is ATPase, pH 9.4?
Light
In type II muscle fibres - what colour is ATPase pH 9.4?
Dark
In the histological appearancde of a normal adult quadricep - what appearance is normal?
Checkerboard
In muscle dystrophies - what level is CK?
200-300 times the normal
In inflammatory myopathy - what level is CK?
20-30 times the normal
In neurogenic disorders - what level is CK?
2-5 times the normal
Give four features of dystrophic muscle changes?
What does increased central nuclei, segmental necrosis, regeneration and ring fibres all suggest?
Dystrophic changes
Are muscular dystrophies inherited?
Yes
Give the inheritance and muscle affected in - Duchenne (DMD)
X-linked recessive
Pelvic girdle
Give the inheritance and muscle affected in - Becker (BMD)
X-linked recessive
Pelvic girdle
Give the inheritance and muscle affected in - Limb girdle (LGMD)
Autosomal recessive
Pelvic girdle
Give the inheritance and muscle affected in - Fascioscapulohumeral
Dominant
Face, shoulder girdle, arm
Give the inheritance and muscle affected in - Scapulohumeral
Autosomal recessive
Shoulder girdle, arm
Give the inheritance and muscle affected in - Oculopharyngeal
Dominant
External ocular and pharynx
Give the inheritance and muscle affected in - Myotonic dystrophy
Dominant
Face, respiratory, limbs
Give 4 pathological features of muscular dystrophies?
When is DMD usually diagnosed?
2-4 years of age (die at 20)
What disease has proximal weakness of quadriceps and pelvic muscles, but pseudohypertrophy of calves - difficulty squatting?
DMD
Describe the mutation in DMD?
Mutations in dystrophin gene on long arm chromosome X