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. 2009;6(1-4):e31-e39.
doi: 10.1016/j.ddmec.2009.02.001.

CARNITINE HOMEOSTASIS, MITOCHONDRIAL FUNCTION, AND CARDIOVASCULAR DISEASE

Shruti Sharma  1 , Stephen M Black
Affiliations

CARNITINE HOMEOSTASIS, MITOCHONDRIAL FUNCTION, AND CARDIOVASCULAR DISEASE

Shruti Sharma et al. Drug Discov Today Dis Mech. 2009.

Abstract

Carnitines are involved in mitochondrial transport of fatty acids and are of critical importance for maintaining normal mitochondrial function. This review summarizes recent experimental and clinical studies showing that mitochondrial dysfunction secondary to a disruption of carnitine homeostasis may play a role in decreased NO signaling and the development of endothelial dysfunction. Future challenges include development of agents that can positively modulate L-carnitine homeostasis which may have high therapeutic potential.

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Figures

Figure 1
Figure 1
Chemical structures of L-carnitine and Acetyl L-carnitine (Panel A) and the Carnitine Biosynthetic Pathway (Panel B). Abbreviations: TML, trimethyl-lysine; TMLD, trimethyl-lysine deoxygenase; HTML, 3-hydroxy-trimethyl-lysine; TMABA, 4-trimethylaminobutyraldehyde; TMABA-DH, 4-timethylaminobutanal dehydrogenase, BBD, butyrobetaine dioxygenase.
Figure 1
Figure 1
Chemical structures of L-carnitine and Acetyl L-carnitine (Panel A) and the Carnitine Biosynthetic Pathway (Panel B). Abbreviations: TML, trimethyl-lysine; TMLD, trimethyl-lysine deoxygenase; HTML, 3-hydroxy-trimethyl-lysine; TMABA, 4-trimethylaminobutyraldehyde; TMABA-DH, 4-timethylaminobutanal dehydrogenase, BBD, butyrobetaine dioxygenase.
Figure 2
Figure 2
The Carnitine Shuttle and role of carnitine in the mitochondrial oxidation of fatty acids. Abbreviations: CPT-I, carnitine palmitoyltransferase-1; CPT-II, carnitine palmitoyltransferase-2; CACT, carnitine acylcarnitine translocase; CrAT, carnitine acetyl transferase; OMM, outer mitochondrial membrane; IMM, inner mitochondrial membrane.
See this image and copyright information in PMC

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