Muscular dystrophies

muscular dystrophy

A group of diseases characterised by progressive degeneration and/or loss of muscle fibres without nervous system involvement. All or nearly all of them have a hereditary origin but details of the type of genetic defect and of the prognosis for the disease vary from type to type. Duchenne muscular dystrophy (pseudohypertrophic muscular dystrophy) is the most common form. It is due to a sex-linked recessive allele and this is expressed as an absence of the protein dystrophin, the disease in boys shows extensive but insufficient muscle fibre reformation from satellite cells.


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