PMID- 22030098
OWN - NLM
STAT- MEDLINE
DCOM- 20120531
LR  - 20220310
IS  - 1096-7206 (Electronic)
IS  - 1096-7192 (Print)
IS  - 1096-7192 (Linking)
VI  - 105
IP  - 1
DP  - 2012 Jan
TI  - Substrate oxidation and cardiac performance during exercise in disorders of long 
      chain fatty acid oxidation.
PG  - 110-5
LID - 10.1016/j.ymgme.2011.09.030 [doi]
AB  - BACKGROUND: The use of long-chain fatty acids (LCFAs) for energy is inhibited in 
      inherited disorders of long-chain fatty acid oxidation (FAO). Increased energy 
      demands during exercise can lead to cardiomyopathy and rhabdomyolysis. 
      Medium-chain triglycerides (MCTs) bypass the block in long-chain FAO and may 
      provide an alternative energy substrate to exercising muscle. OBJECTIVES: To 
      determine the influence of isocaloric MCT versus carbohydrate (CHO) 
      supplementation prior to exercise on substrate oxidation and cardiac workload in 
      participants with carnitine palmitoyltransferase 2 (CPT2), very long-chain 
      acyl-CoA dehydrogenase (VLCAD) and long-chain 3-hydroxyacyl CoA dehydrogenase 
      (LCHAD) deficiencies. DESIGN: Eleven subjects completed two 45-minute, moderate 
      intensity, treadmill exercise studies in a randomized crossover design. An 
      isocaloric oral dose of CHO or MCT-oil was administered prior to exercise; 
      hemodynamic and metabolic indices were assessed during exertion. RESULTS: When 
      exercise was pretreated with MCT, respiratory exchange ratio (RER), steady state 
      heart rate and generation of glycolytic intermediates significantly decreased 
      while circulating ketone bodies significantly increased. CONCLUSIONS: MCT 
      supplementation prior to exercise increases the oxidation of medium chain fats, 
      decreases the oxidation of glucose and acutely lowers cardiac workload during 
      exercise for the same amount of work performed when compared with CHO 
      pre-supplementation. We propose that MCT may expand the usable energy supply, 
      particularly in the form of ketone bodies, and improve the oxidative capacity of 
      the heart in this population.
CI  - Copyright (c) 2011 Elsevier Inc. All rights reserved.
FAU - Behrend, Annie M
AU  - Behrend AM
AD  - Graduate Programs in Human Nutrition, OHSU, USA. behrenda@ohsu.edu
FAU - Harding, Cary O
AU  - Harding CO
FAU - Shoemaker, James D
AU  - Shoemaker JD
FAU - Matern, Dietrich
AU  - Matern D
FAU - Sahn, David J
AU  - Sahn DJ
FAU - Elliot, Diane L
AU  - Elliot DL
FAU - Gillingham, Melanie B
AU  - Gillingham MB
LA  - eng
SI  - ClinicalTrials.gov/NCT00654004
GR  - ULI RR24140/RR/NCRR NIH HHS/United States
GR  - K01 DK071869-04/DK/NIDDK NIH HHS/United States
GR  - K01 DK071869/DK/NIDDK NIH HHS/United States
GR  - K01 NIDDK DK071869/PHS HHS/United States
GR  - UL1 RR024140/RR/NCRR NIH HHS/United States
PT  - Journal Article
PT  - Randomized Controlled Trial
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20111001
PL  - United States
TA  - Mol Genet Metab
JT  - Molecular genetics and metabolism
JID - 9805456
RN  - 0 (Fatty Acids)
RN  - 0 (Ketones)
RN  - 33X04XA5AT (Lactic Acid)
RN  - 6DH1W9VH8Q (Acetylcarnitine)
RN  - 8558G7RUTR (Pyruvic Acid)
RN  - EC 1.3.8.8 (Acyl-CoA Dehydrogenase, Long-Chain)
RN  - EC 2.7.3.2 (Creatine Kinase)
RN  - Long-chain acyl-CoA dehydrogenase deficiency
SB  - IM
MH  - Acetylcarnitine/metabolism
MH  - Acyl-CoA Dehydrogenase, Long-Chain/blood/deficiency/metabolism
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Creatine Kinase/metabolism
MH  - Demography
MH  - Exercise/*physiology
MH  - Fatty Acids/blood/*metabolism
MH  - Female
MH  - Glycolysis
MH  - *Heart Function Tests
MH  - Heart Rate
MH  - Humans
MH  - Ketones/blood
MH  - Lactic Acid/blood
MH  - Lipid Metabolism, Inborn Errors/blood/*metabolism/*physiopathology
MH  - Male
MH  - Oxidation-Reduction
MH  - Oxygen Consumption
MH  - Pyruvic Acid/blood
MH  - Respiration
MH  - Substrate Specificity
MH  - Young Adult
PMC - PMC3253922
MID - NIHMS329808
COIS- Conflict of Interest: The authors have no conflicts of interest relevant to this 
      article to disclose.
EDAT- 2011/10/28 06:00
MHDA- 2012/06/01 06:00
PMCR- 2013/01/01
CRDT- 2011/10/28 06:00
PHST- 2011/08/15 00:00 [received]
PHST- 2011/09/23 00:00 [revised]
PHST- 2011/09/24 00:00 [accepted]
PHST- 2011/10/28 06:00 [entrez]
PHST- 2011/10/28 06:00 [pubmed]
PHST- 2012/06/01 06:00 [medline]
PHST- 2013/01/01 00:00 [pmc-release]
AID - S1096-7192(11)00341-6 [pii]
AID - 10.1016/j.ymgme.2011.09.030 [doi]
PST - ppublish
SO  - Mol Genet Metab. 2012 Jan;105(1):110-5. doi: 10.1016/j.ymgme.2011.09.030. Epub 
      2011 Oct 1.