Schindler disease: An inherited neuroaxonal dystrophy due to α-N-acetylgalactosaminidase deficiency

1990 ◽  
Vol 13 (4) ◽  
pp. 549-559 ◽  
Author(s):  
R. J. Desnick ◽  
A. M. Wang
Keyword(s):  
Neuroaxonal Dystrophy ◽  
Schindler Disease

The Role of Mitochondria in the Genesis of Neuroaxonal Dystrophy in the Brains of Aging Mice

1975 ◽  
Vol 33 ◽  
pp. 372-373
Author(s):  
J.E. Johnson
Keyword(s):  
Electron Microscopy ◽  
Present Report ◽  
Light And Electron Microscopy ◽  
Dorsal Column ◽  
Neuroaxonal Dystrophy ◽  
Nucleus Gracilis ◽  
Dystrophic Axons ◽  
The Brain ◽  
Nucleus Cuneatus

Although neuroaxonal dystrophy (NAD) has been examined by light and electron microscopy for years, the nature of the components in the dystrophic axons is not well understood. The present report examines nucleus gracilis and cuneatus (the dorsal column nuclei) in the brain stem of aging mice.Mice (C57BL/6J) were sacrificed by aldehyde perfusion at ages ranging from 3 months to 23 months. Several brain areas and parts of other organs were processed for electron microscopy.At 3 months of age, very little evidence of NAD can be discerned by light microscopy. At the EM level, a few axons are found to contain dystrophic material. By 23 months of age, the entire nucleus gracilis is filled with dystrophic axons. Much less NAD is seen in nucleus cuneatus by comparison. The most recurrent pattern of NAD is an enlarged profile, in the center of which is a mass of reticulated material (reticulated portion; or RP).

Effects of sorbinil, dietary myo-inositol supplementation, and insulin on resolution of neuroaxonal dystrophy in mesenteric nerves of streptozocin-induced diabetic rats

Diabetes ◽  
1991 ◽  
Vol 40 (5) ◽  
pp. 574-582 ◽  
Author(s):  
R. E. Schmidt ◽  
S. B. Plurad ◽  
B. D. Coleman ◽  
J. R. Williamson ◽  
R. G. Tilton
Keyword(s):  
Diabetic Rats ◽  
Neuroaxonal Dystrophy

Electrophysiological studies in American Quarter horses with neuroaxonal dystrophy

2012 ◽  
Vol 15 ◽  
pp. 3-7 ◽  
Author(s):  
Carrie J. Finno ◽  
Monica Aleman ◽  
Ron Ofri ◽  
Steven R. Hollingsworth ◽  
John E. Madigan ◽  
...  
Keyword(s):  
Neuroaxonal Dystrophy ◽  
Electrophysiological Studies

A case of Schindler disease in the setting of familial cardiomyopathy

2018 ◽  
Vol 123 (2) ◽  
pp. S111
Author(s):  
Damara Ortiz ◽  
Nadene Henderson ◽  
Roxanne Acquaro ◽  
Taraka Donti ◽  
Sara Cathey ◽  
...  
Keyword(s):  
Familial Cardiomyopathy ◽  
Schindler Disease

scholarly journals Inherited neuroaxonal dystrophy in dogs causing lethal, fetal-onset motor system dysfunction and cerebellar hypoplasia

2010 ◽  
Vol 518 (18) ◽  
pp. 3771-3784 ◽  
Author(s):  
John C. Fyfe ◽  
Raba' A. Al-Tamimi ◽  
Rudy J. Castellani ◽  
Diana Rosenstein ◽  
Daniel Goldowitz ◽  
...  
Keyword(s):  
Motor System ◽  
Neuroaxonal Dystrophy ◽  
Cerebellar Hypoplasia
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