Abstract 427: Alterations in the Mitochondrial Supercomplex in Pediatric Dilated Cardiomyopathy

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Kathryn C Chatfield ◽  
Genevieve C Sparagna ◽  
Sarah Chau ◽  
Michael J Bennett ◽  
Adam J Chicco ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Dilated Cardiomyopathy ◽  
Phospholipid Composition ◽  
Tissue Bank ◽  
Fatty Acyl ◽  
Energy Utilization ◽  
Physical Interaction ◽  
Inner Mitochondrial Membrane ◽  
Cross Sectional ◽  
Mitochondrial Fatty Acid

Organization of the mitochondrial electron transport chain (ETC) into a protein “supercomplex” has been shown to be critical for optimal mitochondrial respiration, and is dependent on the phospholipid composition of the inner mitochondrial membrane. A close physical interaction between the ETC supercomplex and the fatty acid beta-oxidation system (FAO, which provides necessary reducing equivalents for ETC) has also been proposed. We have previously demonstrated that content of the primary mitochondrial phospholipid, cardiolipin, is altered in pediatric dilated cardiomyopathy (DCM), with evidence for its dysregulated biosynthesis. We hypothesized that altered cardiolipin content in pediatric DCM is correlated with altered supercomplex-associated ETC activity and mitochondrial fatty acid β-oxidation. A cross-sectional investigation was performed using myocardium from 16 children with DCM and 15 non-failing (NF) controls from the University of Colorado Heart Tissue Bank. Using blue native (BN) -PAGE with in-gel activity staining we demonstrated lower activity of supercomplex-associated complexes I (DCM 80% of NF, P<0.05) and IV (DCM 72% of NF, P<0.05) in pediatric DCM compared with NF controls. Using BN-PAGE and Western blot, as well as proteomic analysis of isolated supercomplex bands, we demonstrated interaction of the ETC supercomplex with FAO enzymes. Quantification of fatty acyl-CoAs was also performed in tissue from pediatric patients with DCM which demonstrated altered content of a subset of acyl-CoAs when compared to NF controls. We detected higher content of some C8, C10 and C12 CoAs in DCM compared with NF (P<0.05), with depletion of C18:1, C18:2, and C16 species (P<0.05). There was no difference between groups in free CoA or Acetyl-CoA. Taken together, these data suggest a potentially important interaction between the ETC supercomplex and long-chain β-oxidation enzymes, which may be altered on heart failure. We provide preliminary evidence for disrupted energy utilization in the failing pediatric heart.

scholarly journals Medium-Chain Acyl-CoA Dehydrogenase Deficiency in an Infant with Dilated Cardiomyopathy

2009 ◽  
Vol 2009 ◽  
pp. 1-3 ◽  
Author(s):  
Marcello Marcì ◽  
Patrizia Ajovalasit
Keyword(s):  
Fatty Acid ◽  
Dilated Cardiomyopathy ◽  
Dehydrogenase Deficiency ◽  
Acid Oxidation ◽  
Inherited Disorders ◽  
Mitochondrial Fatty Acid Oxidation ◽  
Medium Chain ◽  
Mitochondrial Fatty Acid ◽  
Chain Acyl ◽  
Long Chain

We report about an infant affected by dilated cardiomyopathy (CMP) in whom metabolic investigations evidenced medium-chain-acyl-CoA dehydrogenase deficiency (MCADD), that is one of three types of inherited disorders of mitochondrial fatty-acid -oxidation. Long-chain and very long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficits are recognized as responsible of hypertrophic or, less frequently, dilated cardiomyopathy (CMP) in childhood. Otherwise, to our knowledge, no case of MCADD associated to dilated CMP has been reported in literature.

scholarly journals The yeast acyltransferase Sct1p regulates fatty acid desaturation by competing with the desaturase Ole1p

2012 ◽  
Vol 23 (7) ◽  
pp. 1146-1156 ◽  
Author(s):  
Cedric H. De Smet ◽  
Elisa Vittone ◽  
Max Scherer ◽  
Martin Houweling ◽  
Gerhard Liebisch ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Fatty Acid ◽  
Saturated Fatty Acids ◽  
Fatty Acid Desaturase ◽  
Phospholipid Composition ◽  
Fatty Acyl ◽  
Fatty Acid Desaturation ◽  
Growth Defect ◽  
Regulation Of Expression ◽  
Fatty Acid Unsaturation

The degree of fatty acid unsaturation, that is, the ratio of unsaturated versus saturated fatty acyl chains, determines membrane fluidity. Regulation of expression of the fatty acid desaturase Ole1p was hitherto the only known mechanism governing the degree of fatty acid unsaturation in Saccharomyces cerevisiae. We report a novel mechanism for the regulation of fatty acid desaturation that is based on competition between Ole1p and the glycerol-3-phosphate acyltransferase Sct1p/Gat2p for the common substrate C16:0-CoA. Deletion of SCT1 decreases the content of saturated fatty acids, whereas overexpression of SCT1 dramatically decreases the desaturation of fatty acids and affects phospholipid composition. Whereas overexpression of Ole1p increases desaturation, co-overexpression of Ole1p and Sct1p results in a fatty acid composition intermediate between those obtained upon overexpression of the enzymes separately. On the basis of these results, we propose that Sct1p sequesters C16:0-CoA into lipids, thereby shielding it from desaturation by Ole1p. Ta­king advantage of the growth defect conferred by overexpressing SCT1, we identified the acyltransferase Cst26p/Psi1p as a regulator of Sct1p activity by affecting the phosphorylation state and overexpression level of Sct1p. The level of Sct1p phosphorylation is increased when cells are supplemented with saturated fatty acids, demonstrating the physiological relevance of our findings.

Glucocorticoids promote mitochondrial fatty acid oxidation in the fetal heart

2019 ◽  
Author(s):  
Helena Urquijo ◽  
Emma N Panting ◽  
Roderick N Carter ◽  
Emma J Agnew ◽  
Caitlin S Wyrwoll ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Fetal Heart ◽  
Acid Oxidation ◽  
Mitochondrial Fatty Acid Oxidation ◽  
Mitochondrial Fatty Acid

A STUDY ON THE CORRELATION BETWEEN MORPHOLOGIC ANORMALIES AND LEFT VENTRICULAR DIASTOLIC FUNCTION IN DILATED CARDIO-MYOPATHY

2011 ◽  
pp. 137-144
Author(s):  
Thi Ngoc Ha Hoang ◽  
Anh Vu Nguyen ◽  
Minh Loi Hoang ◽  
Cuu Long Nguyen ◽  
Thi Thuy Hang Nguyen
Keyword(s):  
Pulmonary Artery ◽  
Dilated Cardiomyopathy ◽  
Diastolic Dysfunction ◽  
Diastolic Function ◽  
Pulmonary Arteries ◽  
Left Ventricular ◽  
University Hospital ◽  
Cross Sectional ◽  
X Ray ◽  
Key Words Dilated Cardiomyopathy

Purposes: Describe the morphological and diastolic function of left ventricular changes in the patients with dilated cardiomyopathy (DCM) on US, X-ray findings, and Evaluate the correlation between morphology and diastolic function of left ventricular. Materials and method: Cross sectional study from Dec 2009 to Aug 2010, on 39 patients with dilated cardiomyopathy were evaluated at the University Hospital of Hue College of Medical and Pharmaceutical. Results: 1. X-ray and US findings characteristics of DCM is significantly increased in diameter of L, H and mG; LVM, LVMI, LVDd and LAD. 2. The pression of pulmonary artery has been significantly increased with redistribution pulmonary arteries in 61.5% cases and 23.1% have reversed pulmonary artery distribution. 3. DCM have diastolic dysfunction in 100% patients, including severe disorders to 61.5%; the restrictive dysfunction has ratio E/A>2 and E/Em average was 23.89± 17.23. 4.The correlation between the morphology and function in DCM: the diameter of H and L on the X-ray, LAD and ratio LA/AO on US correlated with the level of diastolic dysfunction (p< 0.05). All three radiographic parameters on the radio standard (H, L, the index Cardio/Thoracic) and LVDd on US have negative correlated with EF and FS with p <0.05. Key words: dilated cardiomyopathy, diastolic dysfunction, cardiac tissue Doppler, reversed pulmonary artery distribution

Apoprotein C effect on triglyceride transport in tandem-perfused rat hind end and liver

1984 ◽  
Vol 247 (3) ◽  
pp. G305-G310
Author(s):  
W. J. Kortz ◽  
J. R. Nashold ◽  
M. R. Greenfield ◽  
H. Hilderman ◽  
S. H. Quarfordt
Keyword(s):  
Fatty Acid ◽  
Free Fatty Acid ◽  
Fatty Acyl ◽  
Liver Fat ◽  
Molar Ratio ◽  
Perfusion System ◽  
In Vitro Perfusion ◽  
Arterial Inflow ◽  
High Concentrations

The metabolism of double-labeled triglyceride in a synthetic emulsion was defined in an in vitro perfusion system of rat hind end and liver described previously [Am. J. Physiol. 245 (Gastrointest. Liver Physiol. 8): G106-G112, 1983]. The metabolism of [3H]glycerol-[14C]triolein was defined in the absence of added apoproteins and with additions of human CII and both CII and CIII. Without apoprotein, a pronounced lipolysis of the triglyceride was recognized by high concentrations of radiolabeled glycerol and free fatty acid in the perfusate. The removal of an aliquot of hind-end venous effluent 5 min after adding the labeled triglyceride emulsion to the arterial inflow demonstrated a brisk lipolysis of the substrate when incubated outside the perfusion system. The addition of CII protein to the emulsion before its introduction into the tandem system eliminated perfusate lipolysis, both within the perfusion system and in incubations of aliquots withdrawn from the system. Intravascular lipolysis was not seen with triglyceride emulsions containing both CII and CIH or when an aliquot of hind-end venous effluent was incubated with triglycerides that had not been exposed to the perfusion system. The intravascular lipolysis observed for the [14C]triglyceride added to the tandem system without apoproteins was associated with relatively greater recoveries of 14C-fatty acyl in liver, fat, and muscle and relatively greater recoveries of 14CO2 than when CII alone or both CII and CIII were added with the triglyceride. The addition of CIII to CII in a 1:1 molar ratio increased the recovery of 14C-fatty acyl in muscle and the recovery as 14CO2.(ABSTRACT TRUNCATED AT 250 WORDS)

scholarly journals Quantitation of acyl-CoA and acylcarnitine esters accumulated during abnormal mitochondrial fatty acid oxidation.

1991 ◽  
Vol 266 (34) ◽  
pp. 22932-22938
Author(s):  
R.S. Kler ◽  
S. Jackson ◽  
K. Bartlett ◽  
L.A. Bindoff ◽  
S. Eaton ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Acid Oxidation ◽  
Mitochondrial Fatty Acid Oxidation ◽  
Mitochondrial Fatty Acid

scholarly journals Low-temperature and atmospheric pressure plasma for palm biodiesel hydrogenation

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Grittima Kongprawes ◽  
Doonyapong Wongsawaeng ◽  
Kanokwan Ngaosuwan ◽  
Worapon Kiatkittipong ◽  
Suttichai Assabumrungrat
Keyword(s):  
Fatty Acid ◽  
Cloud Point ◽  
Atmospheric Pressure ◽  
Oxidation Stability ◽  
Acid Methyl Ester ◽  
Energy Utilization ◽  
Superior Performance ◽  
Partial Hydrogenation ◽  
Dbd Plasma ◽  
Plasma Hydrogenation

AbstractPartially hydrogenated fatty acid methyl ester (H-FAME) is conventionally produced through partial hydrogenation under high pressure and elevated temperature in the presence of a catalyst. Herein, a novel green, catalyst-free, non-thermal and atmospheric pressure dielectric barrier discharge (DBD) plasma was employed instead of a conventional method to hydrogenate palm FAME. H-FAME became more saturated with the conversion of C18:2 and C18:3 of 47.4 and 100%, respectively, at 100 W input power, 1 mm gas-filled gap size and 80% H2 in the mixed gas at room temperature for 5 h, causing a reduction of the iodine value from 50.2 to 43.5. Oxidation stability increased from 12.8 to 20 h while a cloud point changed from 13.5 to 16 °C. Interestingly, DBD plasma hydrogenation resulted in no trans-fatty acid formation which provided a positive effect on the cloud point. This green DBD plasma system showed a superior performance to a conventional catalytic reaction. It is an alternative method that is safe from explosion due to the mild operating condition, as well as being highly environmentally friendly by reducing waste and energy utilization from the regeneration process required for a catalytic process. This novel green plasma hydrogenation technique could also be applied to other liquid-based processes.

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