Chemical Formula: C12H23NO5
Chemical Formula C12H23NO5
Found 39 metabolite its formula value is C12H23NO5
Beta-hydroxyisovaleroylcarnitine
3-Hydroxyisovalerylcarnitine is structual derivative of 3-hydroxyisovaleric acid and carnitine. Due to its increased concentration in blood, 3-hydroxyisovalerylcarnitine (C5OH-I) is an important indicator for the diagnosis of organic acidemias in newborns (PMID: 18088573). Analysis of acylcarnitines by tandem mass spectrometry (MS/MS) has recently been used to screen newborns for organic acidemias and fatty acid oxidation defects (PMID: 7494654, 9216448, 11427446, 12127323, 14578311). These diseases cause the accumulation of acyl-CoA, which is esterified to acylcarnitine by carnitine acyltransferase. Acylcarnitine is then eliminated in the urine, thus acylcarnitine concentration serves as an excellent indicator for these diseases (PMID: 6361812). 3-Hydroxyisovalerylcarnitine (C5OH-I) is an indicator for diagnoses of multiple carboxylase deficiency (MCD), 3-methylcrotonyl-CoA carboxylase deficiency and 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (PMID: 18088573), which are inborn errors of metabolism.
hydroxyisovaleroyl carnitine
hydroxyisovaleroyl carnitine is classified as a member of the Acyl carnitines. Acyl carnitines are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. hydroxyisovaleroyl carnitine is considered to be practically insoluble (in water) and acidic. hydroxyisovaleroyl carnitine is a fatty ester lipid molecule
2-Hydroxyisovalerylcarnitine
2-Hydroxyisovalerylcarnitine belongs to the class of organic compounds known as acylcarnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. 2-Hydroxyisovalerylcarnitine is an extremely weak basic (i. e. Human blood levels of 2-hydroxyisovalerylcarnitine were reported to be influenced by genetic variants in the gene HLCS which codes for the enzyme holocarboxylase synthetase (PMID: 26401656). Holocarboxylase synthetase deficiency (OMIM: 253270), also called HLCS deficiency, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder caused by a defective holocarboxylase synthetase. Individuals with HLCS deficiency are unable to utilize biotin effectively.
(2S,3R)-3-Hydroxy-2-methylbutanoylcarnitine
(2S,3R)-3-Hydroxy-2-methylbutanoylcarnitine is an acylcarnitine. More specifically, it is an (2S,3R)-3-hydroxy-2-methylbutanoic acid ester of carnitine. Acylcarnitines were first discovered more than 70 year ago (PMID: 13825279). It is believed that there are more than 1000 types of acylcarnitines in the human body. The general role of acylcarnitines is to transport acyl-groups (organic acids and fatty acids) from the cytoplasm into the mitochondria so that they can be broken down to produce energy. This process is known as beta-oxidation. According to a recent review [Dambrova et al. 2021, Physiological Reviews], acylcarnitines (ACs) can be classified into 9 different categories depending on the type and size of their acyl-group: 1) short-chain ACs; 2) medium-chain ACs; 3) long-chain ACs; 4) very long-chain ACs; 5) hydroxy ACs; 6) branched chain ACs; 7) unsaturated ACs; 8) dicarboxylic ACs and 9) miscellaneous ACs. Short-chain ACs have acyl-groups with two to five carbons (C2-C5), medium-chain ACs have acyl-groups with six to thirteen carbons (C6-C13), long-chain ACs have acyl-groups with fourteen to twenty once carbons (C14-C21) and very long-chain ACs have acyl groups with more than 22 carbons. (2S,3R)-3-Hydroxy-2-methylbutanoylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine (2S,3R)-3-Hydroxy-2-methylbutanoylcarnitine is a member of the most abundant group of carnitines in the body, comprising more than 50\\% of all acylcarnitines quantified in tissues and biofluids (PMID: 31920980). Some short-chain carnitines have been studied as supplements or treatments for a number of diseases, including neurological disorders and inborn errors of metabolism. Carnitine acetyltransferase (CrAT, EC:2.3.1.7) is responsible for the synthesis of all short-chain and short branched-chain acylcarnitines (PMID: 23485643). The study of acylcarnitines is an active area of research and it is likely that many novel acylcarnitines will be discovered in the coming years. It is also likely that many novel roles in health and disease will be uncovered. An excellent review of the current state of knowledge for acylcarnitines is available at [Dambrova et al. 2021, Physiological Reviews].
3-Hydroxyvalerylcarnitine
3-Hydroxyvalerylcarnitine is an acylcarnitine. More specifically, it is an 3-hydroxypentanoic acid ester of carnitine. Acylcarnitines were first discovered more than 70 year ago (PMID: 13825279). It is believed that there are more than 1000 types of acylcarnitines in the human body. The general role of acylcarnitines is to transport acyl-groups (organic acids and fatty acids) from the cytoplasm into the mitochondria so that they can be broken down to produce energy. This process is known as beta-oxidation. According to a recent review [Dambrova et al. 2021, Physiological Reviews], acylcarnitines (ACs) can be classified into 9 different categories depending on the type and size of their acyl-group: 1) short-chain ACs; 2) medium-chain ACs; 3) long-chain ACs; 4) very long-chain ACs; 5) hydroxy ACs; 6) branched chain ACs; 7) unsaturated ACs; 8) dicarboxylic ACs and 9) miscellaneous ACs. Short-chain ACs have acyl-groups with two to five carbons (C2-C5), medium-chain ACs have acyl-groups with six to thirteen carbons (C6-C13), long-chain ACs have acyl-groups with fourteen to twenty once carbons (C14-C21) and very long-chain ACs have acyl groups with more than 22 carbons. 3-Hydroxyvalerylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine 3-Hydroxyvalerylcarnitine is a member of the most abundant group of carnitines in the body, comprising more than 50\\% of all acylcarnitines quantified in tissues and biofluids (PMID: 31920980). Some short-chain carnitines have been studied as supplements or treatments for a number of diseases, including neurological disorders and inborn errors of metabolism. In particular 3-Hydroxyvalerylcarnitine is elevated in the blood or plasma of individuals with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (PMID: 28583327) and holocarboxylase synthetase deficiency (PMID: 27114915). It is also decreased in the blood or plasma of individuals with psoriasis (PMID: 33391503). Carnitine acetyltransferase (CrAT, EC:2.3.1.7) is responsible for the synthesis of all short-chain and short branched-chain acylcarnitines (PMID: 23485643). The study of acylcarnitines is an active area of research and it is likely that many novel acylcarnitines will be discovered in the coming years. It is also likely that many novel roles in health and disease will be uncovered. An excellent review of the current state of knowledge for acylcarnitines is available at [Dambrova et al. 2021, Physiological Reviews].
5-Hydroxypentanoylcarnitine
5-hydroxypentanoylcarnitine is an acylcarnitine. More specifically, it is an 5-hydroxypentanoic acid ester of carnitine. Acylcarnitines were first discovered more than 70 year ago (PMID: 13825279). It is believed that there are more than 1000 types of acylcarnitines in the human body. The general role of acylcarnitines is to transport acyl-groups (organic acids and fatty acids) from the cytoplasm into the mitochondria so that they can be broken down to produce energy. This process is known as beta-oxidation. According to a recent review [Dambrova et al. 2021, Physiological Reviews], acylcarnitines (ACs) can be classified into 9 different categories depending on the type and size of their acyl-group: 1) short-chain ACs; 2) medium-chain ACs; 3) long-chain ACs; 4) very long-chain ACs; 5) hydroxy ACs; 6) branched chain ACs; 7) unsaturated ACs; 8) dicarboxylic ACs and 9) miscellaneous ACs. Short-chain ACs have acyl-groups with two to five carbons (C2-C5), medium-chain ACs have acyl-groups with six to thirteen carbons (C6-C13), long-chain ACs have acyl-groups with fourteen to twenty once carbons (C14-C21) and very long-chain ACs have acyl groups with more than 22 carbons. 5-hydroxypentanoylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine 5-hydroxypentanoylcarnitine is a member of the most abundant group of carnitines in the body, comprising more than 50\\% of all acylcarnitines quantified in tissues and biofluids (PMID: 31920980). Some short-chain carnitines have been studied as supplements or treatments for a number of diseases, including neurological disorders and inborn errors of metabolism. In particular 5-hydroxypentanoylcarnitine is elevated in the blood or plasma of individuals with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (PMID: 28583327) and holocarboxylase synthetase deficiency (PMID: 27114915). It is also decreased in the blood or plasma of individuals with psoriasis (PMID: 33391503). Carnitine acetyltransferase (CrAT, EC:2.3.1.7) is responsible for the synthesis of all short-chain and short branched-chain acylcarnitines (PMID: 23485643). The study of acylcarnitines is an active area of research and it is likely that many novel acylcarnitines will be discovered in the coming years. It is also likely that many novel roles in health and disease will be uncovered. An excellent review of the current state of knowledge for acylcarnitines is available at [Dambrova et al. 2021, Physiological Reviews].
(s)-methyl 4-(boc-(methyl)amino)-5-hydroxypentanoate
N-BOC-(2R,3S)-2-HYDROXY-3-AMINO-5-METHYLHEXANOIC ACID
Boc-(2S,3R)-3-amino-2-hydroxy-5-methylhexanoic acid
(2S,3S)-3-((TERT-BUTOXYCARBONYL)AMINO)-2-HYDROXY-5-METHYLHEXANOIC ACID
1-Piperidinecarboxylic acid, 3-hydroxy-4,4-dimethoxy-, 1,1-dimethylethyl ester
boc-(2r,3r)-3-amino-2-hydroxy-5-methylhexanoic acid
(3S)-3-[(3-hydroxypentanoyl)oxy]-4-(trimethylazaniumyl)butanoate
(R)-3-((3-Hydroxy-3-methylbutanoyl)oxy)-4-(trimethylammonio)butanoate
3-Hydroxy-3-[hydroxy-(trimethylazaniumyl)methyl]-6-methyl-4-oxoheptanoate
Hydroxyisovaleroylcarnitine
An O-acylcarnitine having 3-hydroxyisovaleryl as the acyl substituent.
O-(3-hydroxyvaleryl)-L-carnitine
An O-acyl-L-carnitine in which the acyl group is specified as 3-hydroxyvaleryl.
O-(hydroxyvaleryl)carnitine
An O-acylcarnitine having hydroxyvaleryl as the acyl substituent in which the position of the hydroxy group is unspecified.
O-hydroxyvaleroyl-L-carnitine
An O-acyl-L-carnitine in which the acyl group specified is hydroxyvaleroyl in which the position of the hydroxy group is unspecified.