Skip Navigation

Cardiovascular Research 2002 54(3):516-527; doi:10.1016/S0008-6363(01)00552-1
© 2002 by European Society of Cardiology
This Article
Right arrow Full Text Freely available
Right arrow FREE Full Text (PDF) Freely available
Right arrow E-letters: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when E-letters are posted
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Marín-García, J.
Right arrow Articles by Goldenthal, M. J.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Marín-García, J.
Right arrow Articles by Goldenthal, M. J.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

Copyright © 2002, European Society of Cardiology

Fatty acid metabolism in cardiac failure: biochemical, genetic and cellular analysis

José Marín-García* and Michael J. Goldenthal

The Molecular Cardiology and Neuromuscular Institute, 75 Raritan Avenue, Highland Park, NJ 08904, USA

tmci@att.net

* Corresponding author. Tel.: +1-732-22017-19; fax: +1-732-220-2992

Received 1 October 2001; accepted 22 November 2001

KEYWORDS ACC, acetyl coA carboxylase; ATP, adenosine triphosphate; CPT, carnitine palmitoyl transferase; DCM, dilated cardiomyopathy; ETF, electron-transfer flavoprotein; FADH2, flavin adenine dinucleotide (reduced form); FABP, fatty acid binding protein; FAT, fatty acid translocase; FATP, fatty acid transport protein; HADHA, human mitochondrial trifunctional protein subunit A; HADHB, human mitochondrial trifunctional protein subunit B; HCM, hypertrophic cardiomyopathy; LCAD, long-chain acyl-CoA dehydrogenase; LCHAD, long-chain 3-hydroxyacyl-CoA dehydrogenase; MCAD, medium-chain acyl-CoA dehydrogenase; mtTFA, mitochondrial transcription factor A; MTP, mitochondrial trifunctional protein; NADH, nicotinamide adenine dinucleotide (reduced form); NRF, nuclear regulatory factor; OCTN, organic cation (carnitine) transporter; OXPHOS, oxidative phosphorylation; PGC, nuclear receptor transcriptional co-activator; PPAR, peroxisomal proliferating activating receptors; RXR, retinoid X receptor; SCAD, short-chain acyl-CoA dehydrogenase; TCA, tricarboxylic acid cycle; VLCAD, very long-chain acyl-CoA dehydrogenase

The first 150 words of the full text of this article appear below.


   1 Introduction
 
Despite the abundant literature dealing with the metabolism of fatty acid in the heart, there is a limited understanding (and to the best of our knowledge no comprehensive review) concerning the role that cardiac lipid and fatty acid metabolism plays in the genesis and progression of cardiac failure.

What is presently known is:

(a) Fatty acids and associated lipids play an important role in cardiomyocytes structure and function. There is considerable evidence that in the post-natal and adult mammalian heart, fatty acid β oxidation is the preferred pathway for the energy that is required for efficient cardiac pumping.
(b) Specific defects (either inherited or acquired) in mitochondrial fatty acid metabolism may cause cardiomyopathy and arrhythmias that can lead to cardiac failure.

In this review, we discuss the information available concerning the molecular and cellular basis of fatty acid and lipid metabolic perturbations which can lead to cardiac failure. In this . . . [Full Text of this Article]


   2 Role of fatty acids and their metabolism in the normal cardiomyocyte
 
2.1 Structural and regulatory roles in cardiac cell membranes
2.2 Transporters and carriers
2.3 Bioenergetics of fatty acid oxidation
2.4 Cellular location

   3 Changes occur in fatty acid regulation during cardiac growth and development
 
3.1 Fetal to post-natal transition
3.2 Post-natal to adult
3.3 Aging and senescence

   4 Disorders of fatty acid metabolism affect cardiac structure/function
 
4.1 Specific heritable (inborn) deficiencies in fatty acid metabolism are associated with cardiomyopathy and cardiac failure
4.2 Secondary effects on mitochondrial fatty acid β oxidation: relationship to mitochondrial respiration and OXPHOS
4.3 Fatty acid metabolism defects can be associated with either HCM or DCM
4.4 Abnormalities in fatty acid oxidation lead to cardiac arrhythmias and conduction defects
4.5 Fatty acids and cardiac apoptosis

   5 Molecular players and events in fatty acid related cardiac diseases; genes and modulation of gene expression
 
5.1 MCAD
5.2 VLCAD
5.3 CPT-II
5.4 MTP
5.5 PPAR
5.6 Peroxisome proliferator-activated receptor gamma co-activator (PGC-1)

   6 Animal models of defective fatty acid metabolism and cardiac failure
 

   7 Advances in diagnostics and treatment of fatty acid/cardiac disease
 

Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Am. J. Physiol. Heart Circ. Physiol.Home page
H. Tominaga, H. Katoh, K. Odagiri, Y. Takeuchi, H. Kawashima, M. Saotome, T. Urushida, H. Satoh, and H. Hayashi
Different effects of palmitoyl-L-carnitine and palmitoyl-CoA on mitochondrial function in rat ventricular myocytes
Am J Physiol Heart Circ Physiol, July 1, 2008; 295(1): H105 - H112.
[Abstract] [Full Text] [PDF]

Home page
 Cardiovasc ResHome page
T. Asai, K. Okumura, R. Takahashi, H. Matsui, Y. Numaguchi, H. Murakami, R. Murakami, and T. Murohara
Combined therapy with PPAR{alpha} agonist and L-carnitine rescues lipotoxic cardiomyopathy due to systemic carnitine deficiency
Cardiovasc Res, June 1, 2006; 70(3): 566 - 577.
[Abstract] [Full Text] [PDF]