ISSN:
1573-2665
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Recently a strain of C57 BL/KsJ mice showed a progressive degenerative neurologic manifestation with hepatosplenomegaly, similar to Niemann-Pick disease in humans (Miyawakiet al., 1982). Niemann-Pick disease (McKusick 25720), a rare genetic disorder, is characterized by sphingomyelin accumulation, parti cularly in the reticuloendothelial system, due to a genetic defect of sphingomyelin catabolism. Animal models as an analogue of human Niemann-Pick disease have been found in the Siamese cat (Chrispet al., 1970) and CBA mice (Lyonet al., 1965), but no other reports on these strains have been available. The ethical and scientific limitations of human experimentation have made necessary investigations with appropriate animal sys tems. We report here a new murine model to study a lysosomal storage disorder.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF02179154
