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Carnitine and Derivatives in Health and Disease

A special issue of Nutrients (ISSN 2072-6643). This special issue belongs to the section "Nutrition and Metabolism".

Deadline for manuscript submissions: closed (30 November 2020) | Viewed by 32123

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Guest Editor
Department of Psychobiology, Facultad de Psicología, Universitat de Valencia, Avda. Blasco Ibáñez, 21, 46010 Valencia, Spain
Interests: frailty; cognitive impairment; physical decline; quality of life; diet

Special Issue Information

Dear Colleagues,

Carnitine and its derivatives are nutrients that play an essential role in the transportation of fatty acids across inner mitochondria. The carnitine system is involved in some genetic diseases, energy production, the discharge of accumulated toxic substances, the reduction of chemical damage, the optimization of the processes of muscle tissue repair, and the improvement of physical and mental performance.

The multifaceted mechanisms by which carnitine and its derivatives exert their beneficial effects, such as increased protein synthesis and reduced muscle degradation, represent an important objective of clinical research.

L-carnitine and its derivatives have been proposed as a supplementary treatment to conventional medicine, including liver diseases, diabetes kidney disease, male infertility, sarcopenia, fatigue, neuromuscular disease, and cognitive and memory impairment.

The aim of this Special Issue is to provide information about carnitine and derivatives systems and invite comprehensive reviews, clinical trials, epidemiological analyses, and preclinical studies that further elucidate the relationship between carnitine and related compounds in human health.

Dr. Michele Malaguarnera

Guest Editor

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Keywords

  • Carnitine
  • Diet and dietary supplement
  • Lipid metabolism
  • Oxidative stress
  • Neurodegeneration
  • Fatigue
  • Muscular diseases
  • Metabolic dysfunction
  • Betaines

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Published Papers (5 papers)

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Research

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22 pages, 4594 KiB  
Article
Biological Effects of XyloCore, a Glucose Sparing PD Solution, on Mesothelial Cells: Focus on Mesothelial-Mesenchymal Transition, Inflammation and Angiogenesis
by Valentina Masola, Mario Bonomini, Maurizio Onisto, Pietro Manuel Ferraro, Arduino Arduini and Giovanni Gambaro
Nutrients 2021, 13(7), 2282; https://doi.org/10.3390/nu13072282 - 30 Jun 2021
Cited by 14 | Viewed by 3634
Abstract
Glucose-based solutions remain the most used osmotic agents in peritoneal dialysis (PD), but unavoidably they contribute to the loss of peritoneal filtration capacity. Here, we evaluated at a molecular level the effects of XyloCore, a new PD solution with a low glucose content, [...] Read more.
Glucose-based solutions remain the most used osmotic agents in peritoneal dialysis (PD), but unavoidably they contribute to the loss of peritoneal filtration capacity. Here, we evaluated at a molecular level the effects of XyloCore, a new PD solution with a low glucose content, in mesothelial and endothelial cells. Cell viability, integrity of mesothelial and endothelial cell membrane, activation of mesothelial and endothelial to mesenchymal transition programs, inflammation, and angiogenesis were evaluated by several techniques. Results showed that XyloCore preserves mesothelial and endothelial cell viability and membrane integrity. Moreover XyloCore, unlike glucose-based solutions, does not exert pro-fibrotic, -inflammatory, and -angiogenic effects. Overall, the in vitro evidence suggests that XyloCore could represent a potential biocompatible solution promising better outcomes in clinical practice. Full article
(This article belongs to the Special Issue Carnitine and Derivatives in Health and Disease)
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13 pages, 712 KiB  
Article
Carnitine Serum Levels in Frail Older Subjects
by Giulia Malaguarnera, Vito Emanuele Catania, Claudia Bonfiglio, Gaetano Bertino, Enzo Vicari and Michele Malaguarnera
Nutrients 2020, 12(12), 3887; https://doi.org/10.3390/nu12123887 - 19 Dec 2020
Cited by 12 | Viewed by 3608
Abstract
Frailty is an expression that reconciles and condenses loss of autonomy, both physical and cognitive decline and a wide spectrum of adverse outcomes due to aging. The decrease in physical and cognitive activity is associated with altered mitochondrial function, and energy loss and [...] Read more.
Frailty is an expression that reconciles and condenses loss of autonomy, both physical and cognitive decline and a wide spectrum of adverse outcomes due to aging. The decrease in physical and cognitive activity is associated with altered mitochondrial function, and energy loss and consequently morbidity and mortality. In this cross-sectional study, we evaluated the carnitine levels in frailty status. The mean serum concentrations of total carnitine (TC) were lower in frail elderly subjects than in prefrail ones (p = 0.0006), higher in frail vs. robust subjects (p < 0.0001), and higher in prefrail vs. robust subjects (p < 0.0001). The mean serum concentrations of free carnitine (FC) were lower in frail elderly subjects than in prefrail ones (p < 0.0001), lower in frail vs. robust subjects (p < 0.0001) and lower in prefrail vs. robust subjects (p = 0.0009). The mean serum concentrations of acylcarnitine (AC) were higher in frail elderly subjects than in prefrail ones (p = 0.054) and were higher in pre-frail vs. robust subjects (p = 0.0022). The mean urine concentrations of TC were lower in frail elderly subjects than in prefrail ones (p < 0.05) and lower in frail vs. robust subjects (p < 0.0001). The mean urine concentrations of free carnitine were lower in frail elderly vs. robust subjects (p < 0.05). The mean urine concentrations of acyl carnitines were lower in frail elderly subjects than those in both prefrail (p < 0.0001) and robust subjects (p < 0.0001). Conclusion: high levels of carnitine may have a favorable effect on the functional status and may treat the frailty status in older subjects. Full article
(This article belongs to the Special Issue Carnitine and Derivatives in Health and Disease)
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10 pages, 433 KiB  
Article
Association of Low Serum l-Carnitine Levels with Aortic Stiffness in Patients with Non-Dialysis Chronic Kidney Disease
by Yi-Jen Hsieh, Bang-Gee Hsu, Yu-Hsien Lai, Chih-Hsien Wang, Yu-Li Lin, Chiu-Huang Kuo and Jen-Pi Tsai
Nutrients 2020, 12(10), 2918; https://doi.org/10.3390/nu12102918 - 24 Sep 2020
Cited by 4 | Viewed by 2841
Abstract
l-carnitine (LC) is a co-factor in fatty acid metabolism; its role with respect to aortic stiffness (AS) associated with chronic kidney disease (CKD) was unclear. Our aim was to investigate associations between serum LC levels and AS in patients with non-dialysis CKD [...] Read more.
l-carnitine (LC) is a co-factor in fatty acid metabolism; its role with respect to aortic stiffness (AS) associated with chronic kidney disease (CKD) was unclear. Our aim was to investigate associations between serum LC levels and AS in patients with non-dialysis CKD stage 3–5. The AS patients were those with carotid-femoral pulse wave velocities (cfPWV) >10 m/s; those with cfPWV ≤10 m/s were included as controls. Serum LC was measured by liquid chromatography and mass spectrometry. Of 136 CKD patients, the 44 (32.4%) with AS were older, exhibited higher rates of diabetes, and had elevated diastolic and systolic blood pressures (SBP), elevated fasting glucose levels and lower levels of serum LC compared to controls. Multivariable logistic regression revealed that serum LC (odds ratio [OR] = 0.949, 95% confidence interval [CI] 0.911–0.988, p = 0.011) and age (OR = 1.055, 95% CI 1.013–1.099, p = 0.009) were independent predictors of AS. Multivariable stepwise linear regression revealed significant positive (age and SBP) and negative (serum LC) correlations with cfPWV. The area under the curve of serum LC as a means to predict AS in CKD patients was 0.657 (95% CI 0.571–0.736, p = 0.0009). We concluded that low serum LC is a significant predictor of AS in patients diagnosed with CKD. Full article
(This article belongs to the Special Issue Carnitine and Derivatives in Health and Disease)
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11 pages, 649 KiB  
Communication
Usefulness of Carnitine Supplementation for the Complications of Liver Cirrhosis
by Tatsunori Hanai, Makoto Shiraki, Kenji Imai, Atsushi Suetugu, Koji Takai and Masahito Shimizu
Nutrients 2020, 12(7), 1915; https://doi.org/10.3390/nu12071915 - 29 Jun 2020
Cited by 41 | Viewed by 9966
Abstract
Carnitine is a vitamin-like substance that regulates lipid metabolism and energy production. Carnitine homeostasis is mainly regulated by dietary intake and biosynthesis in the organs, including the skeletal muscle and the liver. Therefore, liver cirrhotic patients with reduced food intake, malnutrition, biosynthetic disorder, [...] Read more.
Carnitine is a vitamin-like substance that regulates lipid metabolism and energy production. Carnitine homeostasis is mainly regulated by dietary intake and biosynthesis in the organs, including the skeletal muscle and the liver. Therefore, liver cirrhotic patients with reduced food intake, malnutrition, biosynthetic disorder, and poor storage capacity of carnitine in the skeletal muscle and liver are more likely to experience carnitine deficiency. In particular, liver cirrhotic patients with sarcopenia are at a high risk for developing carnitine deficiency. Carnitine deficiency impairs the important metabolic processes of the liver, such as gluconeogenesis, fatty acid metabolism, albumin biosynthesis, and ammonia detoxification by the urea cycle, and causes hypoalbuminemia and hyperammonemia. Carnitine deficiency should be suspected in liver cirrhotic patients with severe malaise, hepatic encephalopathy, sarcopenia, muscle cramps, and so on. Importantly, the blood carnitine level does not always decrease in patients with liver cirrhosis, and it sometimes exceeds the normal level. Therefore, patients with liver cirrhosis should be treated as if they are in a state of relative carnitine deficiency at the liver, skeletal muscle, and mitochondrial levels, even if the blood carnitine level is not decreased. Recent clinical trials have revealed the effectiveness of carnitine supplementation for the complications of liver cirrhosis, such as hepatic encephalopathy, sarcopenia, and muscle cramps. In conclusion, carnitine deficiency is not always rare in liver cirrhosis, and it requires constant attention in the daily medical care of this disease. Carnitine supplementation might be an important strategy for improving the quality of life of patients with liver cirrhosis. Full article
(This article belongs to the Special Issue Carnitine and Derivatives in Health and Disease)
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Review

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23 pages, 915 KiB  
Review
Acetyl-L-Carnitine in Dementia and Other Cognitive Disorders: A Critical Update
by Manuela Pennisi, Giuseppe Lanza, Mariagiovanna Cantone, Emanuele D’Amico, Francesco Fisicaro, Valentina Puglisi, Luisa Vinciguerra, Rita Bella, Enzo Vicari and Giulia Malaguarnera
Nutrients 2020, 12(5), 1389; https://doi.org/10.3390/nu12051389 - 12 May 2020
Cited by 58 | Viewed by 11209
Abstract
Several studies explored the effects of acetyl-L-carnitine (ALC) in dementia, suggesting a role in slowing down cognitive decline. Nevertheless, in 2003 a systematic review concluded there was insufficient evidence to recommend a clinical use, although a meta-analysis in the same year showed a [...] Read more.
Several studies explored the effects of acetyl-L-carnitine (ALC) in dementia, suggesting a role in slowing down cognitive decline. Nevertheless, in 2003 a systematic review concluded there was insufficient evidence to recommend a clinical use, although a meta-analysis in the same year showed a significant advantage for ALC for clinical scales and psychometric tests. Since then, other studies have been published; however, a critical review is still lacking. We provide an update of the studies on ALC in primary and secondary dementia, highlighting the current limitations and translational implications. Overall, the role of ALC in dementia is still under debate. The underlying mechanisms may include restoring of cell membranes and synaptic functioning, enhancing cholinergic activity, promoting mitochondrial energy metabolism, protecting against toxins, and exerting neurotrophic effects. The effects of ALC on the gut–liver–brain axis seem to identify the category of patients in which the new insights contribute most to the mechanisms of action of ALC, likely being the liver metabolism and the improvement of hepatic detoxifying mechanisms the primary targets. In this framework, our research group has dealt with this topic, focusing on the ALC-related cross-talk mechanisms. Further studies with homogeneous sample and longitudinal assessment are needed before a systematic clinical application. Full article
(This article belongs to the Special Issue Carnitine and Derivatives in Health and Disease)
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