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Showing 49801 - 49810 of 49827 pathways
SMPDB ID Pathway Chemical Compounds Proteins

SMP0124408

Pw125864 View Pathway
Metabolic

Acylcarnitine (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoylcarnitine

(4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoylcarnitine 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, (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoic 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 (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoyl-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, (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoylcarnitine 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, (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoyl-CoA and L-carnitine. (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoyl-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 (4Z,7Z,10Z,13Z,15E,19Z)-17-hydroxydocosa-4,7,10,13,15,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124409

Pw125865 View Pathway
Metabolic

Acylcarnitine (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoylcarnitine

(4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoylcarnitine 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, (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoic 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 (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoyl-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, (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoylcarnitine 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, (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoyl-CoA and L-carnitine. (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoyl-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 (4Z,7Z,11Z,13Z,16Z,19Z)-10-hydroxydocosa-4,7,11,13,16,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124410

Pw125866 View Pathway
Metabolic

Acylcarnitine (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine

(4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine 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, (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoic 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 (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoyl-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, (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine 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, (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoyl-CoA and L-carnitine. (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoyl-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 (4Z,7E,9E,13Z,16Z,19Z)-11-hydroxydocosa-4,7,9,13,16,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124411

Pw125867 View Pathway
Metabolic

Acylcarnitine (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoylcarnitine

(4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoylcarnitine 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, (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoic 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 (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoyl-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, (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoylcarnitine 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, (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoyl-CoA and L-carnitine. (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoyl-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 (4Z,7Z,10Z,14E,16Z,19Z)-13-hydroxydocosa-4,7,10,14,16,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124412

Pw125868 View Pathway
Metabolic

Acylcarnitine (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoylcarnitine

(4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoylcarnitine 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, (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoic 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 (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoyl-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, (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoylcarnitine 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, (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoyl-CoA and L-carnitine. (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoyl-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 (4Z,7Z,10Z,13Z,17Z,19Z)-16-hydroxydocosa-4,7,10,13,17,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124413

Pw125869 View Pathway
Metabolic

Acylcarnitine (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoylcarnitine

(4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoylcarnitine 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, (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoic 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 (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoyl-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, (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoylcarnitine 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, (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoyl-CoA and L-carnitine. (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoyl-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 (4Z,7Z,10Z,13Z,16E,18E)-20-hydroxydocosa-4,7,10,13,16,18-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124414

Pw125870 View Pathway
Metabolic

Acylcarnitine (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine

(5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine 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,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoic 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,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoyl-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,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoyl-CoA reacts with L-carnitine to form (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine 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,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoyl-CoA and L-carnitine. (5Z,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoyl-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,7Z,10Z,13Z,16Z,19Z)-4-hydroxydocosa-5,7,10,13,16,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124415

Pw125871 View Pathway
Metabolic

Acylcarnitine (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoylcarnitine

(4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoylcarnitine 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, (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoic 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 (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoyl-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, (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoylcarnitine 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, (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoyl-CoA and L-carnitine. (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoyl-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 (4Z,8Z,10Z,13Z,16Z,19Z)-7-hydroxydocosa-4,8,10,13,16,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124416

Pw125872 View Pathway
Metabolic

Acylcarnitine (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoylcarnitine

(4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoylcarnitine 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, (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoic 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 (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoyl-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, (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoyl-CoA reacts with L-carnitine to form (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoylcarnitine 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, (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoyl-CoA and L-carnitine. (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoyl-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 (4E,6E,10Z,13Z,16Z,19Z)-8-hydroxydocosa-4,6,10,13,16,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124417

Pw125873 View Pathway
Metabolic

Acylcarnitine Tricosanoylcarnitine

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