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Showing 49791 - 49800 of 49827 pathways
SMPDB ID Pathway Chemical Compounds Proteins

SMP0124398

Pw125854 View Pathway
Metabolic

Acylcarnitine (7Z,10Z,13E)-docosa-7,10,13-trienoylcarnitine

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

SMP0124399

Pw125855 View Pathway
Metabolic

Acylcarnitine (7E,10E,13E,16E)-3-Hydroxydocosa-7,10,13,16-tetraenoylcarnitine

(7E,10E,13E,16E)-3-Hydroxydocosa-7,10,13,16-tetraenoylcarnitine 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, (7E,10E,13E,16E)-3-hydroxydocosa-7,10,13,16-tetraenoic 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 (7E,10E,13E,16E)-3-hydroxydocosa-7,10,13,16-tetraenoyl-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, (7E,10E,13E,16E)-3-hydroxydocosa-7,10,13,16-tetraenoyl-CoA reacts with L-carnitine to form (7E,10E,13E,16E)-3-hydroxydocosa-7,10,13,16-tetraenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (7E,10E,13E,16E)-3-hydroxydocosa-7,10,13,16-tetraenoylcarnitine 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, (7E,10E,13E,16E)-3-hydroxydocosa-7,10,13,16-tetraenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (7E,10E,13E,16E)-3-hydroxydocosa-7,10,13,16-tetraenoyl-CoA and L-carnitine. (7E,10E,13E,16E)-3-Hydroxydocosa-7,10,13,16-tetraenoyl-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 (7E,10E,13E,16E)-3-hydroxydocosa-7,10,13,16-tetraenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124400

Pw125856 View Pathway
Metabolic

Acylcarnitine (7E,10E,13E,16E,19E)-Docosapentaenoylcarnitine

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

SMP0124401

Pw125857 View Pathway
Metabolic

Acylcarnitine (5Z,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoylcarnitine

(5Z,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoylcarnitine 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,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoic 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,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoyl-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,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoyl-CoA reacts with L-carnitine to form (5Z,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoylcarnitine 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,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoyl-CoA and L-carnitine. (5Z,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoyl-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,8Z,11Z,14Z,17Z)-icosa-5,8,11,14,17-pentaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124402

Pw125858 View Pathway
Metabolic

Acylcarnitine (7Z,10Z,13Z,16Z,19Z)-docosa-7,10,13,16,19-pentaenoylcarnitine

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

SMP0124403

Pw125859 View Pathway
Metabolic

Acylcarnitine (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine

(4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine 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,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoic 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,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoyl-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,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoyl-CoA reacts with L-carnitine to form (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine. 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,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine 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,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoyl-CoA and L-carnitine. (4Z,7Z,10Z,13Z,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoyl-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,16Z)-19,20-dihydroxydocosa-4,7,10,13,16-pentaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124404

Pw125860 View Pathway
Metabolic

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

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

SMP0124405

Pw125861 View Pathway
Metabolic

Acylcarnitine (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine

(5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-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, (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-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 (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-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, (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoyl-CoA reacts with L-carnitine to form (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-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, (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoyl-CoA and L-carnitine. (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-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 (5Z,7S,8E,10Z,13Z,15E,17S,19Z)-7,17-dihydroxydocosa-5,8,10,13,15,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124406

Pw125862 View Pathway
Metabolic

Acylcarnitine (4Z,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-hexaenoylcarnitine

(4Z,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-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,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-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,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-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,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-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,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-hexaenoyl-CoA and L-carnitine. (4Z,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-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,7R,8E,10Z,12E,14E,17S,19Z)-7,16,17-trihydroxydocosa-4,8,10,12,14,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

SMP0124407

Pw125863 View Pathway
Metabolic

Acylcarnitine (4Z,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-hexaenoylcarnitine

(4Z,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-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,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-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,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-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,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-hexaenoyl-CoA reacts with L-carnitine to form (4Z,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-hexaenoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (4Z,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-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,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-hexaenoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (4Z,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-hexaenoyl-CoA and L-carnitine. (4Z,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-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,7S,9E,11E,13Z,15E,17S,19Z)-7,8,17-trihydroxydocosa-4,9,11,13,15,19-hexaenoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.
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