Carnitine

(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate

C7H15NO3 (161.105188)


(R)-carnitine is the (R)-enantiomer of carnitine. It has a role as an antilipemic drug, a water-soluble vitamin (role), a nutraceutical, a nootropic agent and a Saccharomyces cerevisiae metabolite. It is a conjugate base of a (R)-carnitinium. It is an enantiomer of a (S)-carnitine. Constituent of striated muscle and liver. It is used therapeutically to stimulate gastric and pancreatic secretions and in the treatment of hyperlipoproteinemias. L-Carnitine is a metabolite found in or produced by Escherichia coli (strain K12, MG1655). Levocarnitine is a Carnitine Analog. Levocarnitine is a natural product found in Mucidula mucida, Pseudo-nitzschia multistriata, and other organisms with data available. Levocarnitine is an amino acid derivative. Levocarnitine facilitates long-chain fatty acid entry into mitochondria, delivering substrate for oxidation and subsequent energy production. Fatty acids are utilized as an energy substrate in all tissues except the brain. (NCI04) 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 essential factor in fatty acid metabolism in mammals. Its 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. In humans, about 25\\\\\% of carnitine is synthesized in the liver, kidney and brain from the amino acids lysine and methionine. Most of the carnitine in the body comes from dietary sources such as red meat and dairy products. Inborn errors of carnitine metabolism can lead to brain deterioration like that of Reyes syndrome, gradually worsening muscle weakness, Duchenne-like muscular dystrophy and extreme muscle weakness with fat accumulation in muscles. Borurn et al. (1979) describe 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... (-)-Carnitine. CAS Common Chemistry. CAS, a division of the American Chemical Society, n.d. https://commonchemistry.cas.org/detail?cas_rn=541-15-1 (retrieved 2024-06-29) (CAS RN: 541-15-1). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0). L-Carnitine ((R)-Carnitine), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine is an antioxidant. L-Carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3]. L-Carnitine ((R)-Carnitine), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine is an antioxidant. L-Carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3].

   

L-Carnitine

(3R)-3-hydroxy-4-(trimethylazaniumyl)butanoate

C7H15NO3 (161.105188)


Carnitine is a non-essential amino acid and a quaternary ammonium compound. Carnitine is also classified as an alcohol (specifically, a trimethylated carboxy-alcohol). Carnitine exists as one of two stereoisomers (the two enantiomers D-carnitine and L-carnitine. Both are biologically active, but only L-carnitine naturally occurs in animals, and D-carnitine is toxic as it inhibits the activity of the L-form. Carnitine is involved in the metabolism in most mammals, plants, and some bacteria. Carnitine plays a key role in lipid metabolism and beta-oxidation. It is used to transport long-chain fatty acids into the mitochondria to be oxidized for energy production. This is done by forming a long chain acetylcarnitine esters which are then transported by carnitine palmitoyltransferase I and carnitine palmitoyltransferase II. Carnitine also participates in removing products of metabolism from cells. Given its key metabolic roles, carnitine is concentrated in skeletal and cardiac muscle as well as other tissues that metabolize fatty acids as an energy source. A normal 70 kilogram person typically produces 11‚Äì34 mg of carnitine per day. Adults eating mixed diets of red meat and other animal products ingest 60‚Äì180 mg of carnitine per day, while vegans consume about 10‚Äì12 mg per day. Most carnitine obtained from the diet is absorbed in the small intestine before entering the blood.[3] The total body content of carnitine is about 20 grams in a person weighing 70 kilograms, with nearly all of it contained within skeletal muscle cells. Carnitine 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. However, most animals, including humans, make their own carnitine; thus, carnitine cannot be considered to be a vitamin. In certain circumstances, such as methionine deficiency, lysine deficiency, vitamin C deficiency or kidney dialysis, carnitine shortages can 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. In humans, about 25\\\\% of carnitine is synthesized in the liver, kidney, and brain from lysine and methionine. Most of the carnitine in the body comes from dietary sources such as red meat and dairy products. Inborn errors of carnitine metabolism such as Reye‚Äôs syndrome can lead to brain deterioration gradually worsening muscle weakness, Duchenne-like muscular dystrophy, and extreme muscle weakness with fat accumulation in muscles. Carnitine is an essential nutrient for pre-term babies and individuals who are unable to eat a normal diet (e.g. non-ketotic hypoglycemics, kidney dialysis patients) (PMID: 115309). In conditions such as kwashiorkor, cirrhosis, and heart muscle disease (cardiomyopathy) as well as in inborn errors of metabolism such as type IV hyperlipidemia and propionic aciduria, carnitine is essential to life and carnitine supplements are critically important. 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. Carnitine supplements may also be useful in many forms of metabolic liver diseases and heart muscle disease. Hearts undergoing severe arrhythmia quickly deplete their stores of carnitine. Athletes, particularly in Europe, have used carnitine supplements for improved endurance. Carnitine may improve muscle building by improving fat utilization and may even be useful in treating obesity. Carnitine may be of value in treating pregnant women, hypothyroid individuals, and male infertility due to t... Malonyl-carnitin, also known as d,l-carnitine or carnitine chloride, is a member of the class of compounds known as carnitines. Carnitines are organic compounds containing the quaternary ammonium compound carnitine. Malonyl-carnitin is slightly soluble (in water) and a weakly acidic compound (based on its pKa). Malonyl-carnitin can be synthesized from butyrate. Malonyl-carnitin is also a parent compound for other transformation products, including but not limited to, O-sebacoylcarnitine, O-(4,8-dimethylnonanoyl)carnitine, and O-(11-carboxyundecanoyl)carnitine. Malonyl-carnitin can be found in avocado, which makes malonyl-carnitin a potential biomarker for the consumption of this food product. Malonyl-carnitin can be found primarily in blood. L-Carnitine ((R)-Carnitine), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine is an antioxidant. L-Carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3]. L-Carnitine ((R)-Carnitine), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine is an antioxidant. L-Carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3].

   

Oudemansin A

Methyl (5Z)-4-methoxy-2-(methoxymethylidene)-3-methyl-6-phenylhex-5-enoic acid

C17H22O4 (290.1518012)


Oudemansin A is found in mushrooms. Oudemansin A is a metabolite of Oudemansiella mucida (porcelain fungus). Metabolite of Oudemansiella mucida (porcelain fungus). Oudemansin A is found in mushrooms.

   

Carnitine

L-carnitine

C7H15NO3 (161.105188)


L-Carnitine ((R)-Carnitine), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine is an antioxidant. L-Carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3]. L-Carnitine ((R)-Carnitine), a highly polar, small zwitterion, is an essential co-factor for the mitochondrial β-oxidation pathway. L-Carnitine functions to transport long chain fatty acyl-CoAs into the mitochondria for degradation by β-oxidation. L-Carnitine is an antioxidant. L-Carnitine can ameliorate metabolic imbalances in many inborn errors of metabolism[1][2][3].

   

Betaine

2-(trimethylazaniumyl)acetate

C5H11NO2 (117.0789746)


Betaine or trimethylglycine is a methylated derivative of glycine. It functions as a methyl donor in that it carries and donates methyl functional groups to facilitate necessary chemical processes. The donation of methyl groups is important to proper liver function, cellular replication, and detoxification reactions. Betaine also plays a role in the manufacture of carnitine and serves to protect the kidneys from damage. Betaine has also been of interest for its role in osmoregulation. As a drug, betaine hydrochloride has been used as a source of hydrochloric acid in the treatment of hypochlorhydria. Betaine has also been used in the treatment of liver disorders, for hyperkalemia, for homocystinuria, and for gastrointestinal disturbances. (From Martindale, The Extra Pharmacopoeia, 30th Ed, p1341). Betaine is found in many foods, some of which are potato puffs, poppy, hazelnut, and garden cress. Betaine. CAS Common Chemistry. CAS, a division of the American Chemical Society, n.d. https://commonchemistry.cas.org/detail?cas_rn=107-43-7 (retrieved 2024-06-28) (CAS RN: 107-43-7). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

   

Carnitine

γ-Trimethyl-hydroxybutyroβine

C7H15NO3 (161.105188)


An amino-acid betaine that is butanoate substituted with a hydroxy group at position C-3 and a trimethylammonium group at C-4.

   

Mucidin

methyl (2Z,3E,5Z)-2-(methoxymethylidene)-3-methyl-6-phenylhexa-3,5-dienoate

C16H18O3 (258.1255878)


An enoate ester that is the methyl ester of (2E,3Z,5E)-2-(methoxymethylene)-3-methyl-6-phenylhexa-3,5-dienoic acid. D010575 - Pesticides > D005659 - Fungicides, Industrial > D000073739 - Strobilurins D000890 - Anti-Infective Agents > D000935 - Antifungal Agents

   

Strobilurin B

Strobilurin B

C17H19ClO4 (322.0971804)


An enoate ester that is the methyl ester of (2E,3Z,5E)-6-(4-chloro-3-methoxyphenyl)-2-(methoxymethylene)-3-methylhexa-3,5-dienoic acid. D010575 - Pesticides > D005659 - Fungicides, Industrial > D000073739 - Strobilurins D016573 - Agrochemicals

   
   

2-(5-propyloxolan-2-ylidene)cyclopentane-1,3-dione

2-(5-propyloxolan-2-ylidene)cyclopentane-1,3-dione

C12H16O3 (208.1099386)


   

5-(tetradeca-2,4,6-trien-8,10-diyn-1-ylidene)furan-2-one

5-(tetradeca-2,4,6-trien-8,10-diyn-1-ylidene)furan-2-one

C18H16O2 (264.1150236)


   
   

14-(5-oxofuran-2-ylidene)tetradeca-2,8,10,12-tetraen-4,6-diynoic acid

14-(5-oxofuran-2-ylidene)tetradeca-2,8,10,12-tetraen-4,6-diynoic acid

C18H12O4 (292.0735552)


   

methyl 6-(4-chloro-3-methoxyphenyl)-4-methoxy-2-(methoxymethylidene)-3-methylhex-5-enoate

methyl 6-(4-chloro-3-methoxyphenyl)-4-methoxy-2-(methoxymethylidene)-3-methylhex-5-enoate

C18H23ClO5 (354.12339380000003)


   

methyl (2e,3s,4s,5e)-4-methoxy-2-(methoxymethylidene)-6-(4-methoxyphenyl)-3-methylhex-5-enoate

methyl (2e,3s,4s,5e)-4-methoxy-2-(methoxymethylidene)-6-(4-methoxyphenyl)-3-methylhex-5-enoate

C18H24O5 (320.1623654)


   

methyl (2z,3s,4s,5e)-4-methoxy-2-(methoxymethylidene)-6-(4-methoxyphenyl)-3-methylhex-5-enoate

methyl (2z,3s,4s,5e)-4-methoxy-2-(methoxymethylidene)-6-(4-methoxyphenyl)-3-methylhex-5-enoate

C18H24O5 (320.1623654)


   

5-(tetradeca-2,4,6,12-tetraen-8,10-diyn-1-ylidene)furan-2-one

5-(tetradeca-2,4,6,12-tetraen-8,10-diyn-1-ylidene)furan-2-one

C18H14O2 (262.0993744)


   

(2e,8e,10e,12e)-14-[(2z)-5-oxofuran-2-ylidene]tetradeca-2,8,10,12-tetraen-4,6-diynoic acid

(2e,8e,10e,12e)-14-[(2z)-5-oxofuran-2-ylidene]tetradeca-2,8,10,12-tetraen-4,6-diynoic acid

C18H12O4 (292.0735552)


   

methyl 2-(methoxymethylidene)-3-methyl-6-phenylhexa-3,5-dienoate

methyl 2-(methoxymethylidene)-3-methyl-6-phenylhexa-3,5-dienoate

C16H18O3 (258.1255878)


   

(5z)-5-[(2e,4e,6e)-tetradeca-2,4,6-trien-8,10-diyn-1-ylidene]furan-2-one

(5z)-5-[(2e,4e,6e)-tetradeca-2,4,6-trien-8,10-diyn-1-ylidene]furan-2-one

C18H16O2 (264.1150236)


   

methyl (2e,3e,5e)-2-(methoxymethylidene)-6-(4-methoxyphenyl)-3-methylhexa-3,5-dienoate

methyl (2e,3e,5e)-2-(methoxymethylidene)-6-(4-methoxyphenyl)-3-methylhexa-3,5-dienoate

C17H20O4 (288.13615200000004)


   

(5z)-5-[(2e,4e,6e,12e)-tetradeca-2,4,6,12-tetraen-8,10-diyn-1-ylidene]furan-2-one

(5z)-5-[(2e,4e,6e,12e)-tetradeca-2,4,6,12-tetraen-8,10-diyn-1-ylidene]furan-2-one

C18H14O2 (262.0993744)


   

methyl (3e,5e)-2-(methoxymethylidene)-6-(4-methoxyphenyl)-3-methylhexa-3,5-dienoate

methyl (3e,5e)-2-(methoxymethylidene)-6-(4-methoxyphenyl)-3-methylhexa-3,5-dienoate

C17H20O4 (288.13615200000004)


   

methyl (2e,3e,5e)-2-(methoxymethylidene)-3-methyl-6-phenylhexa-3,5-dienoate

methyl (2e,3e,5e)-2-(methoxymethylidene)-3-methyl-6-phenylhexa-3,5-dienoate

C16H18O3 (258.1255878)