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Endothelium as a gatekeeper of fatty acid transport.

ABSTRACT: The endothelium transcends all clinical disciplines and is crucial to the function of every organ system. A critical, but poorly understood, role of the endothelium is its ability to control the transport of energy supply according to organ needs. Fatty acids (FAs) in particular represent a key energy source that is utilized by a number of tissues, but utilization must be tightly regulated to avoid potentially deleterious consequences of excess accumulation, including insulin resistance. Recent studies have identified important endothelial signaling mechanisms, involving vascular endothelial growth factor-B, peroxisome proliferator-activated receptor-?, and apelin, that mediate endothelial regulation of FA transport. In this review, we discuss the mechanisms by which these signaling pathways regulate this key endothelial function.

SUBMITTER: Mehrotra D

PROVIDER: S-EPMC3946743 | biostudies-other | 2014 Feb

REPOSITORIES: biostudies-other

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Endothelium as a gatekeeper of fatty acid transport.

Mehrotra Devi D Wu Jingxia J Papangeli Irinna I Chun Hyung J HJ

Trends in endocrinology and metabolism: TEM 20131203 2

The endothelium transcends all clinical disciplines and is crucial to the function of every organ system. A critical, but poorly understood, role of the endothelium is its ability to control the transport of energy supply according to organ needs. Fatty acids (FAs) in particular represent a key energy source that is utilized by a number of tissues, but utilization must be tightly regulated to avoid potentially deleterious consequences of excess accumulation, including insulin resistance. Recent ...[more]

PMID: 24315207

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Project description:Carnitine is essential for the transfer of long-chain fatty acids across the inner mitochondrial membrane for subsequent β-oxidation. It can be synthesized by the body or assumed with the diet from meat and dairy products. Defects in carnitine biosynthesis do not routinely result in low plasma carnitine levels. Carnitine is accumulated by the cells and retained by kidneys using OCTN2, a high affinity organic cation transporter specific for carnitine. Defects in the OCTN2 carnitine transporter results in autosomal recessive primary carnitine deficiency characterized by decreased intracellular carnitine accumulation, increased losses of carnitine in the urine, and low serum carnitine levels. Patients can present early in life with hypoketotic hypoglycemia and hepatic encephalopathy, or later in life with skeletal and cardiac myopathy or sudden death from cardiac arrhythmia, usually triggered by fasting or catabolic state. This disease responds to oral carnitine that, in pharmacological doses, enters cells using the amino acid transporter B(0,+). Primary carnitine deficiency can be suspected from the clinical presentation or identified by low levels of free carnitine (C0) in the newborn screening. Some adult patients have been diagnosed following the birth of an unaffected child with very low carnitine levels in the newborn screening. The diagnosis is confirmed by measuring low carnitine uptake in the patients' fibroblasts or by DNA sequencing of the SLC22A5 gene encoding the OCTN2 carnitine transporter. Some mutations are specific for certain ethnic backgrounds, but the majority are private and identified only in individual families. Although the genotype usually does not correlate with metabolic or cardiac involvement in primary carnitine deficiency, patients presenting as adults tend to have at least one missense mutation retaining residual activity. This article is part of a Special Issue entitled: Mitochondrial Channels edited by Pierre Sonveaux, Pierre Maechler and Jean-Claude Martinou.

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Endothelium as a gatekeeper of fatty acid transport.

Publications

Endothelium as a gatekeeper of fatty acid transport.

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