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Neuronal changes in GM1 gangliosidosis. A, Late infantile form, 5 years, male. Ballooning of neurons in the frontal cortex. HE stain, ×180. (Courtesy of
Dr. Yoshio Morimatsu, Tokyo Metropolitan Institute of Neuroscience, Fuchu, Japan.) B, Infantile form, 5 years, male. Numerous MCBs in the cytoplasm of
a Purkinje cell in the cerebellum. ×6300. (Courtesy of Dr. Yoshio Morimatsu, Tokyo Metropolitan Institute of Neuroscience, Fuchu, Japan.) C, Adult form.
Head of caudate nucleus. Almost all neurons are markedly distended with Luxol fast blue (LFB)–stained material within the cytoplasm. LFB-HE stain,
×1000. (Courtesy of Dr. Junichi Tanaka, Tokyo Jikei University School of Medicine, Tokyo, Japan.) D, Adult form. Cerebral cortex. Neurons are fairly well
preserved in size, shape, number, and distribution. An occasional distended neuron is visible in the central field. LFB-HE stain, ×500. (Courtesy of Dr.
Source: β-Galactosidase Deficiency (β-Galactosidosis): GM1 Gangliosidosis and Morquio B Disease, The Online Metabolic and Molecular Bases
Junichi Tanaka, Tokyo Jikei University School of Medicine, Tokyo, Japan.)
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 02, 2017
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