Immunohistochemical detection of neural cell adhesion molecule and laminin in X-linked dystrophic dogs and mdx mice.
Although dystrophin deficiency is known to be the genetic and biochemical defect causing Duchenne muscular dystrophy (DMD), much remains unknown about the underlying factors affecting clinical and pathological expression of the disease. Two animal forms of muscular dystrophy resembling DMD have been described. Neural cell adhesion molecule (NCAM) and laminin expression were examined in the proliferation-competent mdx mouse and non-regenerative "golden retriever muscular dystrophy dog" (GRMD). The results showed that (1) NCAM expression was greater in dystrophic dogs and mice than in age-matched normal animals, (2) myoblast-specific NCAM was greater in mdx mice than in dystrophic dogs, and (3) laminin strongly labelled mdx and GRMD myofibre membranes but was also sometimes found in individual interstitial cells of mdx muscle. Expression of these proteins may partly determine the clinicopathological expression of dystrophin deficiency.
College of Veterinary Medicine, North Carolina State University, Raleigh 27606.
S M
SM
Prattis
S B
SB
Horton
S D
SD
van Camp
J N
JN
Kornegay
D000818:Animals; D002353:Carrier State / Q000473:pathology; D015816:Cell Adhesion Molecules, Neuronal / Q000032:analysis*; D004285:Dogs; D005260:Female; D008040:Genetic Linkage; D007150:Immunohistochemistry; D007797:Laminin / Q000032:analysis*; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D009137:Muscular Dystrophy, Animal / Q000235:genetics / Q000473:pathology*; D014960:X Chromosome
D015816:Cell Adhesion Molecules, Neuronal; D007797:Laminin