Sphingomyelinase and nonspecific phosphodiesterase activities in Epstein-Barr virus-transformed lymphoid cell lines from Niemann-Pick disease A, B and C.

Abstract

Acid sphingomyelinase activity determined using the natural substrate, [choline-methyl-14C]sphingomyelin, or the chromogenic synthetic analogue, 2-N-(hexadecanoyl)amino-4-nitrophenylphosphorylcholine, was deficient in Epstein-Barr virus-transformed lymphoid cell lines from Niemann-Pick disease types A and B. In contrast, lines from Niemann-Pick disease type C and "sea-blue histiocyte syndrome" showed a sphingomyelinase activity within the normal range. Bis(4-methylumbelliferyl)phosphate and bis(4-methylumbelliferyl)pyrophosphate phosphodiesterase activities were not deficient in any Niemann-Pick disease cell line. These results demonstrate the validity of such cell lines as an experimental model system for enzymatic studies of Niemann-Pick disease.

• Authors: T Levade, R Salvayre, G Lenoir, L Douste-Blazy
• Journal: Biochimica et biophysica acta (Biochim Biophys Acta) Vol. 793 Issue 2 Pg. 321-4 (Apr 18 1984) ISSN: 0006-3002 [Print] Netherlands
• PMID: 6324871 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Sphingomyelins
• Umbelliferones
• Hymecromone
• bis-(4-methylumbelliferyl)phosphate
• bis(4-methylumbelliferyl)pyrophosphate
• Phosphoric Diester Hydrolases
• Sphingomyelin Phosphodiesterase

Topics

• Adult
• Cell Line
• Cell Transformation, Viral
• Herpesvirus 4, Human
• Humans
• Hymecromone (analogs & derivatives)
• Lymphocytes (enzymology)
• Niemann-Pick Diseases (enzymology)
• Phosphoric Diester Hydrolases (metabolism)
• Sphingomyelin Phosphodiesterase (metabolism)
• Sphingomyelins (metabolism)
• Umbelliferones (metabolism)