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Showing 111381 - 111390 of 111423 pathways
PathBank ID Pathway Chemical Compounds Proteins

SMP0123614

Pw125070 View Pathway
Metabolite

Acylcarnitine 9-methyldodecanoylcarnitine

Homo sapiens
9-methyldodecanoylcarnitine 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, 9-methyldodecanoic 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 9-methyldodecanoyl-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, 9-methyldodecanoyl-CoA reacts with L-carnitine to form 9-methyldodecanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 9-methyldodecanoylcarnitine 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, 9-methyldodecanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 9-methyldodecanoyl-CoA and L-carnitine. 9-methyldodecanoyl-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 9-methyldodecanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123515

Pw124971 View Pathway
Metabolite

Acylcarnitine 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoylcarnitine

Homo sapiens
7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoylcarnitine 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, 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoic 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 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoyl-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, 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoyl-CoA reacts with L-carnitine to form 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoylcarnitine 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, 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoyl-CoA and L-carnitine. 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoyl-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 7-(5-hexyl-3,4-dimethylfuran-2-yl)heptanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123520

Pw124976 View Pathway
Metabolite

Acylcarnitine 7-(3-methyl-5-pentylfuran-2-yl)heptanoylcarnitine

Homo sapiens
7-(3-methyl-5-pentylfuran-2-yl)heptanoylcarnitine 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, 7-(3-methyl-5-pentylfuran-2-yl)heptanoic 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 7-(3-methyl-5-pentylfuran-2-yl)heptanoyl-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, 7-(3-methyl-5-pentylfuran-2-yl)heptanoyl-CoA reacts with L-carnitine to form 7-(3-methyl-5-pentylfuran-2-yl)heptanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 7-(3-methyl-5-pentylfuran-2-yl)heptanoylcarnitine 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, 7-(3-methyl-5-pentylfuran-2-yl)heptanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 7-(3-methyl-5-pentylfuran-2-yl)heptanoyl-CoA and L-carnitine. 7-(3-methyl-5-pentylfuran-2-yl)heptanoyl-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 7-(3-methyl-5-pentylfuran-2-yl)heptanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123527

Pw124983 View Pathway
Metabolite

Acylcarnitine 9-(5-pentylfuran-2-yl)nonanoylcarnitine

Homo sapiens
9-(5-pentylfuran-2-yl)nonanoylcarnitine 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, 9-(5-pentylfuran-2-yl)nonanoic 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 9-(5-pentylfuran-2-yl)nonanoyl-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, 9-(5-pentylfuran-2-yl)nonanoyl-CoA reacts with L-carnitine to form 9-(5-pentylfuran-2-yl)nonanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 9-(5-pentylfuran-2-yl)nonanoylcarnitine 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, 9-(5-pentylfuran-2-yl)nonanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 9-(5-pentylfuran-2-yl)nonanoyl-CoA and L-carnitine. 9-(5-pentylfuran-2-yl)nonanoyl-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 9-(5-pentylfuran-2-yl)nonanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123513

Pw124969 View Pathway
Metabolite

Acylcarnitine 6-(5-hexylfuran-2-yl)hexanoylcarnitine

Homo sapiens
6-(5-hexylfuran-2-yl)hexanoylcarnitine 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, 6-(5-hexylfuran-2-yl)hexanoic 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 6-(5-hexylfuran-2-yl)hexanoyl-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, 6-(5-hexylfuran-2-yl)hexanoyl-CoA reacts with L-carnitine to form 6-(5-hexylfuran-2-yl)hexanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 6-(5-hexylfuran-2-yl)hexanoylcarnitine 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, 6-(5-hexylfuran-2-yl)hexanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 6-(5-hexylfuran-2-yl)hexanoyl-CoA and L-carnitine. 6-(5-hexylfuran-2-yl)hexanoyl-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 6-(5-hexylfuran-2-yl)hexanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123550

Pw125006 View Pathway
Metabolite

Acylcarnitine (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoylcarnitine

Homo sapiens
(5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoylcarnitine 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, (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoic 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 (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoyl-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, (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoyl-CoA reacts with L-carnitine to form (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoylcarnitine 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, (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoyl-CoA and L-carnitine. (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoyl-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 (5Z)-7-[(1S,5E)-5-[(2E)-oct-2-en-1-ylidene]-4-oxocyclopent-2-en-1-yl]hept-5-enoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123545

Pw125001 View Pathway
Metabolite

Acylcarnitine (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoylcarnitine

Homo sapiens
(2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoylcarnitine 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, (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoic 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 (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoyl-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, (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoyl-CoA reacts with L-carnitine to form (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoylcarnitine 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, (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoyl-CoA and L-carnitine. (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoyl-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 (2S)-2-amino-4-{[(1R)-1-[(carboxymethyl)carbamoyl]-2-[(hydroxymethyl)sulfanyl]ethyl]carbamoyl}butanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123539

Pw124995 View Pathway
Metabolite

Acylcarnitine 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoylcarnitine

Homo sapiens
7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoylcarnitine 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, 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoic 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 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoyl-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, 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoyl-CoA reacts with L-carnitine to form 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoylcarnitine 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, 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoyl-CoA and L-carnitine. 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoyl-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 7-{2-[(1E,3S)-3-hydroxyoct-1-en-1-yl]-5-oxocyclopent-1-en-1-yl}heptanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123534

Pw124990 View Pathway
Metabolite

Acylcarnitine 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoylcarnitine

Homo sapiens
3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoylcarnitine 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, 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoic 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 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoyl-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, 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoyl-CoA reacts with L-carnitine to form 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoylcarnitine 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, 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoyl-CoA and L-carnitine. 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoyl-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 3-[(2R)-2-hydroxy-3-methyl-3-[(phosphonooxy)methyl]butanamido]propanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic

SMP0123532

Pw124988 View Pathway
Metabolite

Acylcarnitine 4-phenylbutanoylcarnitine

Homo sapiens
4-phenylbutanoylcarnitine 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, 4-phenylbutanoic 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 4-phenylbutanoyl-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, 4-phenylbutanoyl-CoA reacts with L-carnitine to form 4-phenylbutanoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the 4-phenylbutanoylcarnitine 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, 4-phenylbutanoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form 4-phenylbutanoyl-CoA and L-carnitine. 4-phenylbutanoyl-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 4-phenylbutanoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Metabolic
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