Browsing Pathways

(10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoylcarnitine 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, (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoic 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 (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoyl-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, (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoyl-CoA reacts with L-carnitine to form (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoylcarnitine 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, (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoyl-CoA and L-carnitine. (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoyl-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 (10E,15Z)-9,12,13-trihydroxyoctadeca-10,15-dienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

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

(10E,12Z)-9-hydroxyoctadeca-10,12-dienoylcarnitine 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, (10E,12Z)-9-hydroxyoctadeca-10,12-dienoic 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 (10E,12Z)-9-hydroxyoctadeca-10,12-dienoyl-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, (10E,12Z)-9-hydroxyoctadeca-10,12-dienoyl-CoA reacts with L-carnitine to form (10E,12Z)-9-hydroxyoctadeca-10,12-dienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E,12Z)-9-hydroxyoctadeca-10,12-dienoylcarnitine 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, (10E,12Z)-9-hydroxyoctadeca-10,12-dienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E,12Z)-9-hydroxyoctadeca-10,12-dienoyl-CoA and L-carnitine. (10E,12Z)-9-hydroxyoctadeca-10,12-dienoyl-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 (10E,12Z)-9-hydroxyoctadeca-10,12-dienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

(10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-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, (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-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 (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-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, (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-trienoyl-CoA reacts with L-carnitine to form (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-trienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-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, (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-trienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-trienoyl-CoA and L-carnitine. (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-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 (10E,12E,14E)-9-hydroxy-16-oxooctadeca-10,12,14-trienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

(10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-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, (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-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 (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-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, (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoyl-CoA reacts with L-carnitine to form (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-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, (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoyl-CoA and L-carnitine. (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-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 (10E,12E,14E)-16-hydroxy-9-oxooctadeca-10,12,14-trienoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

(10E)-8-Hydroxydodec-10-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, (10E)-8-hydroxydodec-10-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 (10E)-8-hydroxydodec-10-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, (10E)-8-hydroxydodec-10-enoyl-CoA reacts with L-carnitine to form (10E)-8-hydroxydodec-10-enoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E)-8-hydroxydodec-10-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, (10E)-8-hydroxydodec-10-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E)-8-hydroxydodec-10-enoyl-CoA and L-carnitine. (10E)-8-Hydroxydodec-10-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 (10E)-8-hydroxydodec-10-enoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

(10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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 (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoyl-CoA reacts with L-carnitine to form (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoyl-CoA and L-carnitine. (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-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 (10E)-11-(3,4-dimethyl-5-propylfuran-2-yl)undec-10-enoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

(10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-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 (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-enoyl-CoA reacts with L-carnitine to form (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-enoylcarnitine. This reaction is catalyzed by carnitine O-palmitoyltransferase. This enzyme resides in the mitochondrial outer membrane. While this reaction takes place, the (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-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, (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-enoylcarnitine can react with the carnitine O-palmitoyltransferase 2 enzyme found in the mitochondrial inner membrane to once again form (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-enoyl-CoA and L-carnitine. (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-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 (10E)-11-(3,4-dimethyl-5-pentylfuran-2-yl)undec-10-enoylcarnitine from forming and thereby preventing it from being transported into the mitochondria.

Acute intermittent porphyria (AIP), also called Swedish porphyria, is a rare inborn error of metabolism (IEM) and autosomal dominant disorder of heme biosynthesis caused by a defective HMBS gene. The HMBS gene codes for the protein hydroxymethylbilane synthase (porphobilinogen deaminase) which catalyzes the synthesis of porphobilinogen into hydroxymethylbilane. This disorder is characterized by a large accumulation of 5-aminolevulinic acid or porphobilinogen in both urine and serum. Most patients are asymptomatic between attacks. Symptoms of the disorder include abdominal pain, constipation, vomiting, hypertension, muscle weakness, seizures, delirium, coma, and depression. Treatment involves undertaking a high-carbohydrate diet and, during severe attacks, a glucose 10% infusion. It is estimated that AIP affects 5.9 per 1 000 000 people.

G protein-coupled receptors sense stimuli outside the cell and transmit signals across the plasma membrane. Activation of protein kinase C (PKC) is one of the common signaling pathways. When a class of GPCRs are activated by a ligand, they activate Gq protein to bind GTP instead of GDP. After the Gq becomes active, it activates phospholipase C (PLC) to cleave the membrane lipid phosphatidylinositol 4,5-bisphosphate (PIP2) into inositol 1,4,5-trisphosphate (IP3) and diacyl glycerol (DAG). IP3 can bind Ins3P receptor to open calcium channel by diffusion from cytoplasm to ER. Activated calcium channel will release the calcium from ER into cytoplasm. Calcium can activate the kinase activity of PKC.