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Low and moderate concentrations of lysobisphosphatidic acid in brain and liver of patients affected by some storage diseases
Author(s) -
Kahma Kauko,
Brotherus Jaakko,
Haltia Matti,
Renkonen Ossi
Publication year - 1976
Publication title -
lipids
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.601
H-Index - 120
eISSN - 1558-9307
pISSN - 0024-4201
DOI - 10.1007/bf02532899
Subject(s) - lipidology , clinical chemistry , biology , organelle , biochemistry , phosphate , medicine
The relative amount of lysobisphosphatidic acid (LBPA), known also as bis(monoacylglyceryl)phosphate, among the total phospholipids was analyzed in post mortem samples of brain and liver of patients affected by four storage diseases. In spite of the extensive accumulation of storage lysosomes, none of the samples revealed a highly elevated LBPA content comparable to that found in the liver in Niemann‐Pick disease and in the liver in lipidosis induced by 4,4′‐diethylaminoethoxyhexestrol. We conclude that, although LBPA is often present in high concentration in lysosomes of many types of cells, it is not always a major component of these organelles.