On the specificity of the acetylcholinesterase defect in dystrophic mice.

The tetrameric form of acetylcholinesterase (AChE) in ReJ/129 dystrophic mice was demonstrated to be absent from endplate-poor regions of skeletal muscle but present in endplate-rich regions. Skeletal muscle secreted normal amounts of this form of AChE. Visceral organs had normal amounts and distribution of the AChE molecular forms. These results suggest that the AChE defect in dystrophic mice is limited to skeletal muscle, and the defect does not reflect an abnormality of AChE synthesis but probably reflects an inability to incorporate the enzyme into skeletal muscle membranes.