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Stabilizes the sarcollema protects muscle fibre during contraction
Forms the dystrophin-glycoprotein complex

Duchennes Muscular Dystrophy

X linked recessive
pseudohypertrophy due to accumulation of muscle and fat


Less dangerous form of muscle dystrophy

McArdles Disease

Myophosphorylase deficiency
Accumulation of glycogen
Impaired ability to produce lactic acid
muscle cramps


Autosomal dominant
Dysfunction in muscle membrane
Periodic paralysis
Mytonic dystrophy

Periodic Paralysis

Leaking K+ back into cells
Initial inexciteability and thus paralysis
Myotonic dystrophy: Hyper-excitability and delayed relaxation of muscle

Congenital dystrophy

Inherited defect
Can cause mitochondrial, metabolic, membrane, muscular disorder
Progress slowly

DNA Analysis

Used to investigate disorders with major genetic component
Single gene disorders are easiest to identify
May require blood/ muscle samples
Used to detect DMD and Becker

Muscle Biopsy

Dystrophin labelling -DMD
Enzymes of glycolysis- Mc Ardles
Enzymes of oxidative metabolism

cytochrome C staining

Used to test respiratory chain abnormalities

Succinate dehydrogenase

Marker enzyme for mitochondria


Measure action potential of muscle
Useful to measure alterations in muscle action potential

Plasma CK analysis

Indicated muscle damage
Present in normal muscle after high amounts of eccentric contraction
Catalyses conversion of creatine and ADP to phosphocreatine and ADP

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