Mycotoxin rubratoxin B causes fatty acid oxidation disorder (FAOD) like symptoms in mice

2009 ◽  
Vol 189 ◽  
pp. S165
Author(s):  
Hitoshi Nagashima
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Acid Oxidation ◽  
Fatty Acid Oxidation Disorder

A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women

1999 ◽  
Vol 54 (11) ◽  
pp. 679
Author(s):  
Jamal A. Ibdah ◽  
Michael J. Bennett ◽  
Piero Rinaldo ◽  
Yiwen Zhao ◽  
Beverly Gibson ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Liver Disease ◽  
Pregnant Women ◽  
Fatty Acid Oxidation ◽  
Acid Oxidation ◽  
Fatty Acid Oxidation Disorder

Mitochondrial trifunctional protein deficiency: A severe fatty acid oxidation disorder with cardiac and neurologic involvement

2003 ◽  
Vol 142 (6) ◽  
pp. 684-689 ◽  
Author(s):  
Margarethe E.J. den Boer ◽  
Carlo Dionisi-Vici ◽  
Anupam Chakrapani ◽  
Anders O.J. van Thuijl ◽  
Ronald J.A. Wanders ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Protein Deficiency ◽  
Acid Oxidation ◽  
Fatty Acid Oxidation Disorder ◽  
Neurologic Involvement ◽  
Mitochondrial Trifunctional Protein

3-Hydroxydicarboxylic and 3-ketodicarboxylic aciduria in three patients: evidence for a new defect in fatty acid oxidation at the level of 3-ketoacyl-CoA thiolase

1993 ◽  
Vol 39 (5) ◽  
pp. 897-901 ◽  
Author(s):  
M J Bennett ◽  
W G Sherwood
Keyword(s):  
Mass Spectrometry ◽  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Mass Spectra ◽  
Gas Chromatography Mass Spectrometry ◽  
Acid Oxidation ◽  
Fatty Acid Oxidation Disorder ◽  
Urinary Organic Acids ◽  
First Time ◽  
Long Chain

Abstract Three patients presented with evidence of a fatty acid oxidation disorder. Analysis of urinary organic acids by gas chromatography/mass spectrometry demonstrated the presence of medium-chain (C6-C12) dicarboxylic, 3-hydroxydicarboxylic, and 3-ketodicarboxylic acids in all three urines. 3-Ketodicarboxylic aciduria is reported for the first time here, as are the mass spectra for 3-ketosuberic, 3-ketosebacic, and 3-ketododecanedioic acids and the oximated spectrum for 3-ketoadipic acid. The presence of 3-ketodicarboxylic acids suggests a defect at the level of a long-chain 3-ketoacyl-CoA thiolase, an enzyme for which a deficiency state has not previously been described. Our patients may represent the first cases of a long-chain thiolase defect.

scholarly journals Child Neurology: Recurrent rhabdomyolysis due to a fatty acid oxidation disorder

Neurology ◽  
2013 ◽  
Vol 82 (1) ◽  
pp. e1-e4 ◽  
Author(s):  
G. Terrone ◽  
M. Ruoppolo ◽  
N. Brunetti-Pierri ◽  
C. Cozzolino ◽  
E. Scolamiero ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Acid Oxidation ◽  
Fatty Acid Oxidation Disorder ◽  
Child Neurology ◽  
Recurrent Rhabdomyolysis

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: A severe fatty acid oxidation disorder

1994 ◽  
Vol 153 (10) ◽  
pp. 745-750 ◽  
Author(s):  
A. C. Sewell ◽  
S. W. Bender ◽  
S. Wirth ◽  
H. Münterfering ◽  
L. Ijlist ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Dehydrogenase Deficiency ◽  
Acid Oxidation ◽  
Fatty Acid Oxidation Disorder ◽  
Hydroxyacyl Coa Dehydrogenase ◽  
Long Chain

A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women

1999 ◽  
Vol 340 (22) ◽  
pp. 1723-1731 ◽  
Author(s):  
Jamal A. Ibdah ◽  
Michael J. Bennett ◽  
Piero Rinaldo ◽  
Yiwen Zhao ◽  
Beverly Gibson ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Liver Disease ◽  
Pregnant Women ◽  
Fatty Acid Oxidation ◽  
Acid Oxidation ◽  
Fatty Acid Oxidation Disorder
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