'Oil globules' in Duchenne muscular dystrophy--history, demonstration, and metabolic significance.
Duchenne, Meryon, Wernich, Clarke, Down and other dystrophy pioneers recognized, illustrated and/or described Duchenne muscular dystrophy skeletal muscle's 'oil globules'. Rarely mentioned or acknowledged since the introduction in 1869 by Klebs of paraffin embedding and modern histological technique (in which tissue lipids are eliminated) this microscopical marker of metabolic dysfunction is utilized to find its metabolic site of origin in the living cell, to identify the disease's major dysfunctioning metabolic pathway, and finally to determine its dystrophin connection which accounts for the primary metabolic malfunction and the clinical manifestations of disease. This paper presents a working hypothesis developed through a long-term empirical study and suggests a practical method of therapy.
Bonsett
C A
CA
Rudman
A
A
D016189:Dystrophin / Q000378:metabolism; D049672:History, 19th Century; D006801:Humans; D050356:Lipid Metabolism; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology*; D009136:Muscular Dystrophies / Q000266:history / Q000378:metabolism / Q000473:pathology*
D016189:Dystrophin