A role for mast cells in the progression of Duchenne muscular dystrophy?: Correlations in dystrophin-deficient humans, dogs, and mice
JRM Gorospe, MD Tharp, J Hinckley… - Journal of the …, 1994 - Elsevier
Dystrophin deficiency has been shown to be the underlying cause of Duchenne muscular
dystrophy. Although dystrophin-deficient homologous animal models have been identified
(dog, mouse, and cat), the clinical expression of the biochemical defect is species-specific.
Thus, while the genetics and biochemistry of Duchenne dystrophy is understood, the
pathophysiological cascade leading to muscle weakness in only humans and dogs remains
obscure. To begin to dissect the pathophysiology at the histological level, we undertook a …
dystrophy. Although dystrophin-deficient homologous animal models have been identified
(dog, mouse, and cat), the clinical expression of the biochemical defect is species-specific.
Thus, while the genetics and biochemistry of Duchenne dystrophy is understood, the
pathophysiological cascade leading to muscle weakness in only humans and dogs remains
obscure. To begin to dissect the pathophysiology at the histological level, we undertook a …