NIEMANN PICK DISEASE: PRESENCE OF THE MAGNESIUM‐DEPENDENT SPHINGOMYELINASE IN BRAIN OF THE INFANTILE FORM OF THE DISEASE

S. Gatt, T. Dinur, J. Kopolovic

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Abstract

Abstract— Brain of a 14‐month‐old patient with the infantile form of Niemann Pick disease was found to be practically devoid of sphingomyelinase activity when assayed at pH 5. When assayed at pH 7.4, in the presence of magnesium ions considerable hydrolysis of sphingomyelin was obtained by brain preparations. The rate of hydrolis of a homogenate of grey matter was about 0.3 μmol. mg protein−1. H−1, corresponding to about 15 μmol of sphingomyelin hydrolysed perg brain in 1 h. The possibility is suggested that the presence of the extra‐lysosomal, magnesium dependent sphingomyelinase in brain of Niemann Pick patients may be responsible, in part, for the lesser accumulation of sphingomyelin in this tissue relative to other organs, such as liver or spleen.

Original languageEnglish
Pages (from-to)547-550
Number of pages4
JournalJournal of Neurochemistry
Volume31
Issue number2
DOIs
StatePublished - Aug 1978
Externally publishedYes

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