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The Niemann‐Pick Mouse: Sphingomyelinase and phosphodiesterase assay on clipped tail.
Author(s) -
Sakiyama T.,
Tsuda M.,
Nakabayashi H.,
Kitagawa T.
Publication year - 1983
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1983.tb00582.x
Subject(s) - sphingomyelin , sphingomyelin phosphodiesterase , acid sphingomyelinase , medicine , phosphodiesterase , niemann–pick disease , pyrophosphatase , pyrophosphatases , enzyme , biochemistry , disease , cholesterol , biology
In the newly discovered Niemann‐Pick affected mice, sphingomyelinase and phosphodiesterase activities in clipped tail homogemates failed to help determine the genetic status of this disease, which is characterized by a sphingomyelinase deficiency. The cause for this failure might be the existence of neutral sphingomyelinase which contributed to a wide scatter of the results. The age of the specimens probably had no effect on those results. Finally, during tail enzyme analysis, Bis‐(4MU)‐pyrophosphatase activity in an affected liver showed different characteristics than a normal liver.