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Tunica muscularis of the small intestine of an α-N-acetylgalactosaminidase-deficient mouse. A ganglion of the myenteric plexus occupies the center of the
micrograph (triangles). Abnormal storage is seen in several cell types: myenteric plexus neurons contain numerous single-membrane-bound laminated
whorls admixed with electron-dense bodies. Similar inclusions are present in the cytoplasm of smooth muscle cells (asterisks). Interstitial fibroblasts
(arrows) contain many secondary lysosomes distended by flocculent filamentous and vesicular material. Scale bar, 2 μm.
Source: α-N-Acetylgalactosaminidase Deficiency: Schindler
Disease, The Online Metabolic and Molecular Bases of Inherited Disease
Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular
Bases of Inherited Disease; 2014 Available at: http://mhmedical.com/ Accessed: May 10, 2017
Copyright © 2017 McGraw-Hill Education. All rights reserved
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