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PMID: 948249 Published · ppublish English Journal Article

Low and moderate concentrations of lysobisphosphatidic acid in brain and liver of patients affected by some storage diseases.

Lipids ·Vol. 11 ·No. 7 ·1976-07-00 ·Pages 539-44

Kahma K, Brotherus J, Haltia M, Renkonen O

Abstract

The relative amount of lysobisphosphatidic acid (LBPA), known also as bis(monoacylglycerly)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 evelated 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.

MeSH Terms
Adolescent Adult Brain/metabolism Child Child, Preschool Female Humans Lipid Metabolism, Inborn Errors/metabolism,pathology Liver/metabolism,pathology Male Phosphatidic Acids/metabolism Phospholipids/metabolism
Chemicals
Phosphatidic Acids Phospholipids
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Kahma K
Brotherus J
Haltia M
Renkonen O
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24 references, click to expand
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Article Info
Journal
Lipids
Abbr.
Lipids
ISSN
0024-4201
DOI
10.1007/BF02532899
Published
1976-07-00
Pages
539-44
Language
English
Region
United States
NLM ID
0060450
Subset
IM
External Links
PubMed source DOI: 10.1007/BF02532899 View journal details
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