Results 111 to 120 of about 5,834 (144)
Some of the next articles are maybe not open access.
Archives of Neurology, 1974
Lipopigments are prominent in Batten disease (Batten-Spielmeyer-Vogt [BSV] syndrome) and are considered to represent end products of lipid peroxidation. Peroxidase is an enzyme that can hydrolyze peroxides (hydrogen peroxide) and by this means reduce lipid peroxidation.
D, Armstrong, S, Dimmitt, D E, VanWormer
openaire +2 more sources
Lipopigments are prominent in Batten disease (Batten-Spielmeyer-Vogt [BSV] syndrome) and are considered to represent end products of lipid peroxidation. Peroxidase is an enzyme that can hydrolyze peroxides (hydrogen peroxide) and by this means reduce lipid peroxidation.
D, Armstrong, S, Dimmitt, D E, VanWormer
openaire +2 more sources
Cellular distribution of lesions in Batten disease
American Journal of Medical Genetics, 1995AbstractOne of the characteristic manifestations of chronic neuronal lipofuscinosis (Batten disease) is a marked predisposition for epileptic seizures. The management of these seizures is very difficult. The present study was initiated to determine what mechanisms could account for the seizure disorder. Tissue was examined from a patient with a history
R, Chronister +3 more
openaire +2 more sources
Detection of canine batten disease with the EEG
Electroencephalography and Clinical Neurophysiology, 1977Although it has been suggested that EEG changes appear early in the course of human Batten disease, these observations have been made only after the onset of clinical abnormalities and without immediate pathological correlation. In this brief report we have been able to document for the first time, abnormal EEGs in a strain of dogs proposed as a model ...
P, Burcar +5 more
openaire +2 more sources
Pathogenesis of brain dysfunction in Batten disease
American Journal of Medical Genetics, 1995AbstractAnimal models of Batten disease and other neuronal storage disorders offer important opportunities to study the pathogenesis of brain dysfunction in this family of diseases. Although all of these conditions exhibit progressive intraneuronal storage, we have found that other aspects of the cellular pathology of Batten disease differ markedly ...
S U, Walkley +4 more
openaire +2 more sources
Batten Disease and Mitochondrial Pathways of Proteolysis
Biochemical and Molecular Medicine, 1996Most forms of Batten Disease (BD), a group of neurodegenerative diseases, are characterized by the accumulation within lysosomes of the very hydrophobic protein subunit 9 of the mitochondrial F1F0-ATP synthase (F-ATPase). It is now known that the cause of the accumulation of this protein in BD is a reduction in its rate of degradation.
A J, Tanner, J F, Dice
openaire +2 more sources
Cell, 1995
Batten disease (also known as juvenile neuronal ceroid lipofuscinosis) is a recessively inherited neurodegenerative disorder of childhood characterized by progressive loss of vision, seizures, and psychomotor disturbances. The Batten disease gene, CLN3, maps to chromosome 16p12.1.
openaire +2 more sources
Batten disease (also known as juvenile neuronal ceroid lipofuscinosis) is a recessively inherited neurodegenerative disorder of childhood characterized by progressive loss of vision, seizures, and psychomotor disturbances. The Batten disease gene, CLN3, maps to chromosome 16p12.1.
openaire +2 more sources
Gait phenotype in Batten disease: A marker of disease progression
European Journal of Paediatric Neurology, 2021John R Østergaard
exaly