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PW124952

Pw124952 View Pathway
metabolic

Acylcarnitine 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoylcarnitine

Homo sapiens
11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoylcarnitine is an acylcarnitine. 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. As part of this process, 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoyl-CoA reacts with L-carnitine to form 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoyl-CoA and L-carnitine. 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 11-{3,4-dimethyl-5-[(1E)-prop-1-en-1-yl]furan-2-yl}undecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124953

Pw124953 View Pathway
metabolic

Acylcarnitine 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine

Homo sapiens
11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine is an acylcarnitine. 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. As part of this process, 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA reacts with L-carnitine to form 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA and L-carnitine. 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 11-(5-butyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124954

Pw124954 View Pathway
metabolic

Acylcarnitine 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoylcarnitine

Homo sapiens
11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoylcarnitine is an acylcarnitine. 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. As part of this process, 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoyl-CoA reacts with L-carnitine to form 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoyl-CoA and L-carnitine. 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 11-{3,4-dimethyl-5-[(1E)-pent-1-en-1-yl]furan-2-yl}undecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124955

Pw124955 View Pathway
metabolic

Acylcarnitine 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine

Homo sapiens
11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine is an acylcarnitine. 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. As part of this process, 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA reacts with L-carnitine to form 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA and L-carnitine. 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 11-(5-hexyl-3,4-dimethylfuran-2-yl)undecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124956

Pw124956 View Pathway
metabolic

Acylcarnitine 11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine

Homo sapiens
11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine is an acylcarnitine. 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. As part of this process, 11-(5-heptyl-3-methylfuran-2-yl)undecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 11-(5-heptyl-3-methylfuran-2-yl)undecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 11-(5-heptyl-3-methylfuran-2-yl)undecanoyl-CoA reacts with L-carnitine to form 11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 11-(5-heptyl-3-methylfuran-2-yl)undecanoyl-CoA and L-carnitine. 11-(5-heptyl-3-methylfuran-2-yl)undecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 11-(5-heptyl-3-methylfuran-2-yl)undecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124957

Pw124957 View Pathway
metabolic

Acylcarnitine 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoylcarnitine

Homo sapiens
12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoylcarnitine is an acylcarnitine. 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. As part of this process, 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoyl-CoA reacts with L-carnitine to form 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoyl-CoA and L-carnitine. 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 12-(3,4-dimethyl-5-propylfuran-2-yl)dodecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124958

Pw124958 View Pathway
metabolic

Acylcarnitine 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoylcarnitine

Homo sapiens
12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoylcarnitine is an acylcarnitine. 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. As part of this process, 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoyl-CoA reacts with L-carnitine to form 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoyl-CoA and L-carnitine. 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 12-(3,4-dimethyl-5-pentylfuran-2-yl)dodecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124959

Pw124959 View Pathway
metabolic

Acylcarnitine 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoylcarnitine

Homo sapiens
13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoylcarnitine is an acylcarnitine. 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. As part of this process, 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoyl-CoA reacts with L-carnitine to form 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoyl-CoA and L-carnitine. 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 13-(3,4-dimethyl-5-propylfuran-2-yl)tridecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124960

Pw124960 View Pathway
metabolic

Acylcarnitine 13-(3-methyl-5-propylfuran-2-yl)tridecanoylcarnitine

Homo sapiens
13-(3-methyl-5-propylfuran-2-yl)tridecanoylcarnitine is an acylcarnitine. 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. As part of this process, 13-(3-methyl-5-propylfuran-2-yl)tridecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 13-(3-methyl-5-propylfuran-2-yl)tridecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 13-(3-methyl-5-propylfuran-2-yl)tridecanoyl-CoA reacts with L-carnitine to form 13-(3-methyl-5-propylfuran-2-yl)tridecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 13-(3-methyl-5-propylfuran-2-yl)tridecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 13-(3-methyl-5-propylfuran-2-yl)tridecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 13-(3-methyl-5-propylfuran-2-yl)tridecanoyl-CoA and L-carnitine. 13-(3-methyl-5-propylfuran-2-yl)tridecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 13-(3-methyl-5-propylfuran-2-yl)tridecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

PW124961

Pw124961 View Pathway
metabolic

Acylcarnitine 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoylcarnitine

Homo sapiens
15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoylcarnitine is an acylcarnitine. 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. As part of this process, 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoic acid is first transported into the cell via the long-chain fatty acid transport protein 1 (FATP1). Once inside the cell it undergoes a reaction to form an acyl-CoA derivative called 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoyl-CoA. This reaction is facilitated by the long-chain fatty-acid CoA ligase 1 protein, which adds a CoA moiety to appropriate acyl groups. Many acyl-CoA groups will then further react with other zwitterionic compounds such as carnitine (to form acylcarnitines) and amino acids (to form acyl amides). The carnitine needed to form acylcarnitines inside the cell is transported into the cell by the organic cation/carnitine transporter 2. In forming an acylcarnitine derivative, 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoyl-CoA reacts with L-carnitine to form 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoylcarnitine is moved into the mitochondrial intermembrane space. Following the reaction, the newly synthesized acylcarnitine is transported into the mitochondrial matrix by a mitochondrial carnitine/acylcarnitine carrier protein found in the mitochondrial inner membrane. Once in the matrix, 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoyl-CoA and L-carnitine. 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoyl-CoA then enters into the mitochondrial beta-oxidation pathway to form aceytl-CoA. Acetyl-CoA can go on to enter the TCA cycle, or it can react with L-carnitine to form L-acetylcarnitine in a reaction catalyzed by Carnitine O-acetyltransferase. This reaction can occur in both directions, and L-acetylcarnitine and CoA can react to form acetyl-CoA and L-carnitine in certain circumstances. Finally, acetyl-CoA in the cytosol can be catalyzed by acetyl-CoA carboxylase 1 to form malonyl-CoA, which inhibits the action of carnitine O-palmitoyltransferase 1, thereby preventing 15-(3,4-dimethyl-5-propylfuran-2-yl)pentadecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.