Dolichol metabolism in cultured skin fibroblasts from patients with “neuronal” ceroid lipofuscinosis (Batten's disease)

1984 ◽  
Vol 7 (3) ◽  
pp. 112-116 ◽  
Author(s):  
B. C. Paton ◽  
A. Poulos
Keyword(s):  
Neuronal Ceroid Lipofuscinosis ◽  
Skin Fibroblasts ◽  
Ceroid Lipofuscinosis ◽  
Cultured Skin ◽  
Batten's Disease

Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease)

1993 ◽  
Vol 33 (4) ◽  
pp. 381-385 ◽  
Author(s):  
Roderick T. Bronson ◽  
Brian D. Lake ◽  
Susan Cook ◽  
Susanne Taylor ◽  
Muriel T. Davisson
Keyword(s):  
Motor Neuron ◽  
Neuronal Ceroid Lipofuscinosis ◽  
Motor Neuron Degeneration ◽  
Neuron Degeneration ◽  
Ceroid Lipofuscinosis ◽  
Batten's Disease

NEURONAL CEROID-LIPOFUSCINOSIS (BATTEN'S DISEASE): RELATIONSHIP TO AMAUROTIC FAMILY IDIOCY?

PEDIATRICS ◽  
1969 ◽  
Vol 44 (4) ◽  
pp. 570-583
Author(s):  
Wolfgang Zeman ◽  
Paul Dyken
Keyword(s):  
Clinical Data ◽  
Neuronal Ceroid Lipofuscinosis ◽  
The Other ◽  
Gm2 Gangliosidosis ◽  
Gm1 Gangliosidosis ◽  
Ceroid Lipofuscinosis ◽  
Intractable Seizures ◽  
Tay Sachs Disease ◽  
Batten's Disease ◽  
Ceroid Lipofuscin

From the conditions generically classified as amaurotic familial idiocy, two distinctly different groups have emerged. One is characterized by grossly abnormal profiles for cerebral sphingolipids and consists of GM1-gangliosidosis and GM2-gangliosidosis or Tay-Sachs disease. The other group is composed of cases with normal sphingolipid profiles, but with neuronal accumulation of lipopigments of the ceroid/lipofuscin type, designated as neuronal ceroid-lipofuscinosis (NCL) or Batten's disease. Clinical data on 26 patients with this disorder reveal the course and symptomatology to be variable, but pathomorphologic and biochemical findings are reasonably constant. The course of the disorder is directly influenced by the presence or absence of intractable seizures.

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