Production and disposal of medium-chain fatty acids in children with medium-chain acyl-CoA dehydrogenase deficiency

1994 ◽  
Vol 17 (1) ◽  
pp. 74-80 ◽  
Author(s):  
S. J. R. Heales ◽  
G. N. Thompson ◽  
A. F. Massoud ◽  
S. Rahman ◽  
D. Halliday ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Dehydrogenase Deficiency ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Chain Acyl ◽  
Chain Fatty Acids

scholarly journals Population Screening for Medium-Chain Acyl-CoA Dehydrogenase Deficiency: Analysis of Medium-Chain Fatty Acids and Acylglyeines in Blood Spots

1994 ◽  
Vol 31 (1) ◽  
pp. 72-77 ◽  
Author(s):  
Michael J Bennett ◽  
Marie C Ragni ◽  
Robert J Ostfeld ◽  
Rene Santer ◽  
Eberhard Schmidt-Sommerfeld
Keyword(s):  
Fatty Acids ◽  
Dehydrogenase Deficiency ◽  
Decenoic Acid ◽  
Mcad Deficiency ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Blood Spots ◽  
Normal Ranges ◽  
Chain Acyl ◽  
Chain Fatty Acids

We have developed methods for the measurement of the medium-chain fatty acids octanoate, decanoate and cis-4-decenoate and the acylglycines n-hexanoylglycine (HG) and 3-phenylpropionylglycine (PPG) in blood spots using gas chromatography and mass spectrometry. Normal ranges were obtained for octanoate and decanoate. HG, PPG and cis-4-decenoic acid were not detected in control blood spots. In blood spots from nine patients (including two newborn) with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency, all metabolites were present in elevated concentrations although PPG was close to the detection limits and there was overlap for octanoate and decanoate. The lack of detection of cis-4-decenoic acid and HG in controls suggests that these are the metabolites of choice for blood spot identification of infants with MCAD deficiency.

scholarly journals Medium-chain acyl-CoA dehydrogenase, a gatekeeper of mitochondrial function in glioblastoma multiforme

2020 ◽  
Author(s):  
Francesca Puca ◽  
Fei Yu ◽  
Caterina Bertolacci ◽  
Piergiorgio Pettazzoni ◽  
Alessandro Carugo ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Drug Targets ◽  
Feedback Regulation ◽  
Normal Brain ◽  
Negative Feedback Regulation ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Lipid Species ◽  
Chain Acyl ◽  
Chain Fatty Acids

SUMMARYGlioblastoma (GBM) is among the deadliest of human cancers. Despite extensive efforts, it has proven to be highly resistant to chemo- and immune-based therapeutic strategies, and little headway has been made with targeted inhibitors. Like many cancers, metabolism is dysregulated in GBM. Thus, to identify new vulnerabilities and drug targets in GBM, we conducted genetic screens using pooled RNAi libraries targeting metabolic enzymes. We screened multiple glioma stem cell-derived (GSC) xenograft models, which revealed that several enzymes involved in the mitochondrial metabolism of fatty acids were required for tumor cell proliferation. From among these, we focused on medium-chain acyl-CoA dehydrogenase (MCAD), which oxidizes medium-chain fatty acids, due to its consistently high score across all of our screens, as well as its high expression level in multiple GSC models and its upregulation in GBM compared to normal brain.In this manuscript, we describe the dependence of GBM on sustained fatty acid metabolism to actively catabolize lipid species that would otherwise damage the mitochondrial structure. The uptake of mediumchain fatty acids lacks negative feedback regulation; therefore, in the absence of MCAD, medium-chain fatty acids accumulate to toxic levels, inducing reactive oxygen species (ROS), mitochondrial damage and failure, and apoptosis. Taken together, our findings uncover a previously unappreciated protective role exerted by MCAD in GBM cells, making it a unique and therapeutically exploitable vulnerability.

Sudden Child Death and `Healthy' Affected Family Members With Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

PEDIATRICS ◽  
1986 ◽  
Vol 78 (6) ◽  
pp. 1052-1057
Author(s):  
Marinus Duran ◽  
Marchinus Hofkamp ◽  
William J. Rhead ◽  
Jean-Marie Saudubray ◽  
Sybe K. Wadman
Keyword(s):  
Fatty Acids ◽  
Coenzyme A ◽  
Family Members ◽  
Reye Syndrome ◽  
Dicarboxylic Aciduria ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Chain Acyl ◽  
Acyl Coenzyme A ◽  
Chain Fatty Acids

A family is described in which the father and three (and probably all four) of his children had a decreased capacity for the oxidation of medium-chain fatty acids. One of the children suddenly died at the age of 16 months following an episode of a rapidly deteriorating Reye syndrome-like illness with hypoketotic hypoglycemia and dicarboxylic aciduria, but without any previous alarming symptoms. The eldest sibling had died at the age of 19 months under similar conditions. The other family members had always been healthy. On fasting, all affected family members accumulated in their plasma the medium-chain fatty acids octanoic, decanoic, and cis-4-decenoic acids. Their urinary organic acid excretion profile could be characterized as "dicarboxylic aciduria." A deficiency of medium-chain acyl-coenzyme A dehydrogenase was demonstrated in a postmortem liver sample of the index patient. Cultured fibroblasts from the father and the two healthy children had a decreased rate of [14C]octanoate oxidation. It is suggested that a deficiency of medium-chain acyl-coenzyme A dehydrogenase may lead to a life-threatening illness when other complicating factors such as diarrhea and vomiting result in an abnormal depletion of the body's glycogen stores. Careful monitoring of at-risk patients during a minor illness is necessary.

Enhanced Bioavailability of Curcumin Nanoemulsions Stabilized with Phosphatidylcholine Modified with Medium Chain Fatty Acids

2017 ◽  
Vol 14 (3) ◽  
pp. 377-385 ◽  
Author(s):  
Angelica A. Ochoa-Flores ◽  
Josafat A. Hernández-Becerra ◽  
Adriana Cavazos-Garduño ◽  
Ida Soto-Rodríguez ◽  
Maria Guadalupe Sanchez-Otero ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Chain Fatty Acids

scholarly journals Effects of ketogenic diet containing medium-chain fatty acids on serum inflammatory factor and mTOR signaling pathway in rats

2020 ◽  
Vol 7 (1) ◽  
Author(s):  
Huan Liu ◽  
Jingwei Huang ◽  
Hui Liu ◽  
Feng Li ◽  
Quansheng Peng ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Caloric Restriction ◽  
Ketogenic Diet ◽  
Mtor Pathway ◽  
Inflammatory Factor ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Sd Rats ◽  
Anti Inflammatory ◽  
Chain Fatty Acids

Abstract Background The ketogenic diet (KD) can promote the anti-inflammatory metabolic state and increase ketone body level in rats. This study was to explore the effects and differences of KD with or without medium-chain fatty acids (MCFAs) on serum inflammatory factors and mTOR pathway in Sprague–Dawley (SD) rats. Results Male SD rats were assigned to five groups: control diet (C), 20% caloric restriction diet (LC), 20% caloric restriction ketogenic diet (containing MCFAs) (LCKD1), 20% caloric restriction ketogenic diet (LCKD2) and 20% caloric restriction foreign ketogenic diet (LCKD3), and fed for 30 d. LC and KD could significantly reduce the body weight of rats; LC and KD containing MCFAs showed anti-inflammatory effects; KD without MCFAs decreased the concentration of mTOR1, while KD containing MCFAs decreased the expression of AMPK, mtor1 and P70sk. Conclusions KD containing MCFAs showed better effects on the mTOR pathway and anti-inflammation than that without MCFAs.

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