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Role of carnitine esters in brain neuropathology.
Mol Aspects Med. 2004 Oct-Dec; 25(5-6):533-49.MA

Abstract

L-Carnitine (L-C) is a naturally occurring quaternary ammonium compound endogenous in all mammalian species and is a vital cofactor for the mitochondrial oxidation of fatty acids. Fatty acids are utilized as an energy substrate in all tissues, and although glucose is the main energetic substrate in adult brain, fatty acids have also been shown to be utilized by brain as an energy substrate. L-C also participates in the control of the mitochondrial acyl-CoA/CoA ratio, peroxisomal oxidation of fatty acids, and the production of ketone bodies. Due to their intrinsic interaction with the bioenergetic processes, they play an important role in diseases associated with metabolic compromise, especially mitochondrial-related disorders. A deficiency of carnitine is known to have major deleterious effects on the CNS. Several syndromes of secondary carnitine deficiency have been described that may result from defects in intermediary metabolism and alterations principally involving mitochondrial oxidative pathways. Mitochondrial superoxide formation resulting from disturbed electron transfer within the respiratory chain may affect the activities of respiratory chain complexes I, II, III, IV, and V and underlie some CNS pathologies. This mitochondrial dysfunction may be ameliorated by L-C and its esters. In addition to its metabolic role, L-C and its esters such as acetyl-L-carnitine (ALC) poses unique neuroprotective, neuromodulatory, and neurotrophic properties which may play an important role in counteracting various disease processes. Neural dysfunction and metabolic imbalances underlie many diseases, and the inclusion of metabolic modifiers may provide an alternative and early intervention approach, which may limit further developmental damage, cognitive loss, and improve long-term therapeutic outcomes. The neurophysiological and neuroprotective actions of L-C and ALC on cellular processes in the central and peripheral nervous system show such effects. Indeed, many studies have shown improvement in processes, such as memory and learning, and are discussed in this review.

Authors+Show Affiliations

Scientific Affairs, Sigma-tau HealthScience, Pomezia 00040, Italy. ashraf.virmani@st-hs.itNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

15363640

Citation

Virmani, Ashraf, and Zbigniew Binienda. "Role of Carnitine Esters in Brain Neuropathology." Molecular Aspects of Medicine, vol. 25, no. 5-6, 2004, pp. 533-49.
Virmani A, Binienda Z. Role of carnitine esters in brain neuropathology. Mol Aspects Med. 2004;25(5-6):533-49.
Virmani, A., & Binienda, Z. (2004). Role of carnitine esters in brain neuropathology. Molecular Aspects of Medicine, 25(5-6), 533-49.
Virmani A, Binienda Z. Role of Carnitine Esters in Brain Neuropathology. Mol Aspects Med. 2004 Oct-Dec;25(5-6):533-49. PubMed PMID: 15363640.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Role of carnitine esters in brain neuropathology. AU - Virmani,Ashraf, AU - Binienda,Zbigniew, PY - 2004/9/15/pubmed PY - 2005/10/29/medline PY - 2004/9/15/entrez SP - 533 EP - 49 JF - Molecular aspects of medicine JO - Mol Aspects Med VL - 25 IS - 5-6 N2 - L-Carnitine (L-C) is a naturally occurring quaternary ammonium compound endogenous in all mammalian species and is a vital cofactor for the mitochondrial oxidation of fatty acids. Fatty acids are utilized as an energy substrate in all tissues, and although glucose is the main energetic substrate in adult brain, fatty acids have also been shown to be utilized by brain as an energy substrate. L-C also participates in the control of the mitochondrial acyl-CoA/CoA ratio, peroxisomal oxidation of fatty acids, and the production of ketone bodies. Due to their intrinsic interaction with the bioenergetic processes, they play an important role in diseases associated with metabolic compromise, especially mitochondrial-related disorders. A deficiency of carnitine is known to have major deleterious effects on the CNS. Several syndromes of secondary carnitine deficiency have been described that may result from defects in intermediary metabolism and alterations principally involving mitochondrial oxidative pathways. Mitochondrial superoxide formation resulting from disturbed electron transfer within the respiratory chain may affect the activities of respiratory chain complexes I, II, III, IV, and V and underlie some CNS pathologies. This mitochondrial dysfunction may be ameliorated by L-C and its esters. In addition to its metabolic role, L-C and its esters such as acetyl-L-carnitine (ALC) poses unique neuroprotective, neuromodulatory, and neurotrophic properties which may play an important role in counteracting various disease processes. Neural dysfunction and metabolic imbalances underlie many diseases, and the inclusion of metabolic modifiers may provide an alternative and early intervention approach, which may limit further developmental damage, cognitive loss, and improve long-term therapeutic outcomes. The neurophysiological and neuroprotective actions of L-C and ALC on cellular processes in the central and peripheral nervous system show such effects. Indeed, many studies have shown improvement in processes, such as memory and learning, and are discussed in this review. SN - 0098-2997 UR - https://www.unboundmedicine.com/medline/citation/15363640/Role_of_carnitine_esters_in_brain_neuropathology_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0098299704000482 DB - PRIME DP - Unbound Medicine ER -