Carnitine

From NuGOwiki

The NuGOwiki Metabolite Database is a joint initiative of NuGO and HMDB

Taxonomy Navigation Box; search by

Carnitine
2D structure for Carnitine
Chemical Name	(3R)-3-hydroxy-4-trimethylammonio-butanoate
Chemical Formula	C₇H₁₅NO₃
CAS Number	541-15-1
Chemical Information	HMDB00062
Biochemical Taxonomy	Carnitines
Functional Taxonomy	Essential Amino Acid
Nutritional Taxonomy	Not Available
Metabolic Pathways	Alanine and Aspartate Metabolism Lysine Degradation
Biofluid Location	Blood Cerebrospinal Fluid (CSF) Urine
Tissue Location	Bladder Brain Erythrocyte Fibroblasts Intestine Kidney Liver Lung Muscle Myocardium Nerves Neurons Platelet Skeletal Muscle Sperm Testes Testis Adipose Tissue
Normal Biofluid Concentrations	Blood: 33.6 +/- 6.2 uM Blood: 38.2 +/- 5.4 uM Blood: 43.0 (26.0-79.0) uM Cerebrospinal Fluid (CSF): 4.0 (2.0 - 9.0) uM Urine: 18.0 +/- 1.2 umol/mmol creatinine Urine: 23.5 +/- 4.0 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Diabetic Ketosis Lung Cancer
Other (Monogenic Disorders)	Acyl-coa dehydrogenase, medium-chain, deficiency of OMIM: 201450 Carnitine deficiency, systemic primary OMIM: 212140 Carnitine palmitoyltransferase i deficiency OMIM: 255120
Abnormal Biofluid Concentrations	Urine (Diabetic Ketosis): 13.3 (11.6-15.1) umol/mmol creatinine Urine (Diabetic Ketosis): 41.3 (38.3-44.2) umol/mmol creatinine Urine (Lung Cancer): 7.0 (0.0-30.0) umol/mmol creatinine
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

Authors:
Affiliations:

1 Introduction
2 Biological Function
3 Catabolism
4 Diseases / Conditions Related to Nutrition
- 4.1 Associated decreased protein/metabolite profile
- 4.2 Associated increased protein/metabolite profile
5 Other (Monogenic) Disorders
6 Nutritional Information
7 Drivers for biological variation
8 Vulnerable groups
9 Related Links:
10 Other resources
11 Links

Introduction

guidelines
Carnitine is not an essential amino acid; it can be synthesized in the body. However, it is so important in providing energy to muscles including the heart-that some researchers are now recommending carnitine supplements in the diet, particularly for people who do not consume much red meat, the main food source for carnitine. carnitine has been described as a vitamin, an amino acid, or a metabimin, i.e., an essential metabolite. Like the B vitamins, carnitine contains nitrogen and is very soluble in water, and to some researchers carnitine is a vitamin (Liebovitz 1984). It was found that an animal (yellow mealworm) could not grow without carnitine in its diet. However, as it turned out, almost all other animals, including humans, do make their own carnitine; thus, it is no longer considered a vitamin. Nevertheless, in certain circumstances-such as deficiencies of methionine, lysine or vitamin C or kidney dialysis--carnitine shortages develop. Under these conditions, carnitine must be absorbed from food, and for this reason it is sometimes referred to as a "metabimin" or a conditionally essential metabolite. Like the other amino acids used or manufactured by the body, carnitine is an amine. But like choline, which is sometimes considered to be a B vitamin, carnitine is also an alcohol (specifically, a trimethylated carboxy-alcohol). Thus, carnitine is an unusual amino acid and has different functions than most other amino acids, which are most usually employed by the body in the construction of protein. carnitine is an important amino acid made reliance first capital by the body from lysine. It's most important known metabolic function is to transport fat into the mitochondria of muscle cells, including those in the heart, for oxidation. This is how the heart gets most of its energy. Inborn errors of carnitine metabolism can lead to brain deterioration like that of Reye's syndrome, gradually worsening muscle weakness, Duchenne-like muscular dystrophy and extreme muscle weakness with fat accumulation in muscles. Borurn et al. (1979) have summed up the research by describing carnitine as an essential nutrient for pre-term babies, certain types (non-ketotic) of hypoglycemics, kidney dialysis patients, cirrhosis, and in kwashiorkor, type IV hyperlipidemia, heart muscle disease (cardiomyopathy), and propionic or organic aciduria (acid urine resulting from genetic or other anomalies). In all these conditions and the inborn errors of carnitine metabolism, carnitine is essential to life and carnitine supplements are valuable. carnitine therapy may also be useful in a wide variety of clinical conditions. carnitine supplementation has improved some patients who have angina secondary to coronary artery disease. It may be worth a trial in any form of hyperlipidemia or muscle weakness. carnitine supplements may be useful in many forms of toxic or metabolic liver disease and in cases of heart muscle disease. Hearts undergoing severe arrhythmia quickly deplete their stores of carnitine. Athletes, particularly in Europe, have used carnitine supplements for silver price improved endurance. carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. carnitine joins a long list of nutrients which may be of value in treating pregnant women, hypothyroid individuals, and male infertility due to low motility of sperm. Even the Physician's Desk Reference gives indication for carnitine supplements as "improving the tolerance of ischemic heart disease, myocardial insufficiencies, and type IV hyperlipoproteinemia. carnitine deficiency is noted in abnormal liver function, renal dialysis patients, and severe to moderate Chetan Kapur muscular weakness with associated anorexia." (http://www.dcnutrition.com)

Carnitine, also known as L-carnitine (levocarnitine) is a quaternary ammonium compound derived from the amino acid lysine. L-carnitine is a cofactor required for transformation of free long-chain fatty acids into acylcarnitines, and for their subsequent transport into the mitochondrial matrix, where they undergo beta-oxidation for cellular energy production. The most significant source of L-carnitine in human nutrition is meat, although humans can synthesize L-carnitine from dietary amino acids (lysine or methionine). Vitamin C (ascorbic acid) is essential to the synthesis of carnitine.

Constituent of striated muscle and liver. It is used therapeutically to stimulate gastric and pancreatic secretions and in the treatment of hyperlipoproteinemias.