scholarly journals Odd-Numbered Long-Chain Fatty Acids (OLCFA) in Erythrocyte Membrane Lipids as Follow-up Parameter in Propionic Acidemia 165

1996 ◽  
Vol 40 (3) ◽  
pp. 542-542
Author(s):  
T. Meissner ◽  
E. Mayatepek
Neurology ◽  
1986 ◽  
Vol 36 (6) ◽  
pp. 791-791 ◽  
Author(s):  
K. Tanaka ◽  
M. Shimada ◽  
T. Naruto ◽  
H. Yamamoto ◽  
Y. Saeki ◽  
...  

1989 ◽  
Vol 31 (2) ◽  
pp. 136-143 ◽  
Author(s):  
Kazuhiko Tanaka ◽  
Morimi Shimada ◽  
Toshiyuki Naruto ◽  
Hisashi Yamamoto ◽  
Kashirou Nishizawa ◽  
...  

Author(s):  
Thomas Meissner ◽  
Michael Leichsenring ◽  
Ertan Mayatepek

AbstractOdd-numbered long-chain fatty acids (OLCFAs) increase in total erythrocyte lipids or plasma in patients with propionic acidemia and have been proposed as a useful parameter of metabolic control. However, up to now no parameter for long-term metabolic control has been available for this disorder. In contrast to previous investigations, we investigated OLCFAs with particular reference to differences for two phospholipid fractions of erythrocyte lipids. The phosphatidylethanolamine (PE) fraction of erythrocyte lipids has a slow turnover rate and might reflect the metabolic propionyl-CoA burden over a period of some weeks. Distinct increases in OLCFA of up to 200% in the phosphatidylcholine fraction were observed after episodes of metabolic decompensation and maximum OLCFA levels were found 3 weeks after the onset of a crisis. As a sign of poor metabolic control, OLCFA levels in PE steadily increased after recurrent episodes of decompensation, but not in clinically stable patients. However, in one patient with clinically good metabolic control, OLCFAs continually increased after the second year of life for an as yet unknown reason. In conclusion, data from this study indicate the usefulness of OLCFA analysis in the PE fraction of erythrocyte lipids, in particular with regard to the need for such a parameter for evidence-based improvement of management strategies in propionic acidemia.


1998 ◽  
Vol 22 (Supplement 1) ◽  
pp. 103 ◽  
Author(s):  
Junko Adachi ◽  
Atsuko Miwa ◽  
Yasuhiro Ueno ◽  
Migiwa Asano ◽  
Takeaki Naito ◽  
...  

Neurology ◽  
1984 ◽  
Vol 34 (11) ◽  
pp. 1499-1499 ◽  
Author(s):  
Y. Antoku ◽  
T. Sakai ◽  
I. Goto ◽  
H. Iwashita ◽  
Y. Kuroiwa

1981 ◽  
Vol 36 (3) ◽  
pp. 1046-1049 ◽  
Author(s):  
Shoji Tsuji ◽  
Minoru Suzuki ◽  
Toshio Ariga ◽  
Michiko Sekine ◽  
Masaru Kuriyama ◽  
...  

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