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Publication : Neuraminidase deficiency in SM/J mice.

First Author  Womack JE Year  1979
Journal  Mouse News Lett Volume  61
Pages  64 Mgi Jnum  J:13824
Mgi Id  MGI:62003 Citation  Womack JE, et al. (1979) Neuraminidase deficiency in SM/J mice. Mouse News Lett 61:64
abstractText  Full text of MNL contribution: Research News: Neuraminidase deficiency in SM/J mice. (In collaboration with D. Lu Shun Yan, Hopital Sainte - Justine, Universite de Montreal, Montreal, Quebec). Liver from strain SM/J mice is known to express unique electrophoretic patterns of acid phosphatase (Lalley and Shows, 1977, Genetics 87: 305-317), alpha-mannosidase (Dizik and Elliot, 1978, Biochem. Genet. 16: 247-260), arylsulfatase B (Daniel et al, Genetics, in press), and alpha-glucosidase (Peters and Swallow, 1979, MNL 60:46). Tissues other than liver may also express the variant pattern but generally kidney does not. Single genes involved in the processing of these enzymes have been implicated and mapped on chr 17. Since variant electrophoretic patterns of several of these enzymes have been converted to typical patterns by in vitro incubation of SM/J liver homgenates with neuraminidase, an inherited deficiency of neuraminidase activity in SM/J with pleiotropic effects on a number of lysosomal enzymes becomes a viable hypothesis. Utilizing the fluorometric assay developed by Potier (1979, Anal. Biochem. 94: 287-296), with 4methylumbelliferyl - alphaD-N-acetylneuraminic acid as substrate, we have measured levels of activity in livers from strains A/J, AKR/J, BALB/cJ, C3H/HeJ, C57BL/6J, C57BR/cdJ, C57L/J, CBA/J, CE/J, DBA/2J, KK/Wo, P/J, PL/J, RF/J, SJL/J, SK/Cam, SM/J, SWR/J, 129/J, and incipient inbred strains of molossinus and castaneus. Strain SM/J is uniquely deficient with less than 15% of the activity of C57BL/6J liver. The congenic strain B10.SM(70NS)/Sn (SM/J H-2 complex back- crossed onto C57BL/10Sn) is also deficient, imputing genetic responsibility for the deficiency to chr 17. (Womack and Potier)
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