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PathWhiz ID Pathway Meta Data

PW000074

Pw000074 View Pathway
disease

5-Oxoprolinuria

Homo sapiens
5-Oxoprolinuria (5-Oxoprolinase deficiency) is a result of a defect in the gamma-glutamyl cycle due to either 5-oxoprolinase or glutathione synthetase deficiency. In the case of glutathione synthetase deficiency, the glycine is not incorporated into gamma-glutamylcysteine. In the case of 5-oxoprolinase, however, pyroglutamic acid accumulates. Symptoms include anemia, mental retardation, metabolic acidosis, respiratory distress and urolithiasis.

PW127179

Pw127179 View Pathway
disease

5-Oxoprolinuria

Homo sapiens
5-Oxoprolinuria (5-Oxoprolinase deficiency) is a result of a defect in the gamma-glutamyl cycle due to either 5-oxoprolinase or glutathione synthetase deficiency. In the case of glutathione synthetase deficiency, the glycine is not incorporated into gamma-glutamylcysteine. In the case of 5-oxoprolinase, however, pyroglutamic acid accumulates. Symptoms include anemia, mental retardation, metabolic acidosis, respiratory distress and urolithiasis.

PW121697

Pw121697 View Pathway
disease

5-Oxoprolinuria

Mus musculus
5-Oxoprolinuria (5-Oxoprolinase deficiency) is a result of a defect in the gamma-glutamyl cycle due to either 5-oxoprolinase or glutathione synthetase deficiency. In the case of glutathione synthetase deficiency, the glycine is not incorporated into gamma-glutamylcysteine. In the case of 5-oxoprolinase, however, pyroglutamic acid accumulates. Symptoms include anemia, mental retardation, metabolic acidosis, respiratory distress and urolithiasis.

PW127181

Pw127181 View Pathway
disease

5-Oxoprolinase Deficiency

Homo sapiens
5-Oxoprolinase deficiency, also called OPLAHD, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of glutathione metabolism caused by a defective 5-oxoprolinase. 5-Oxoprolinase catalyzes the conversion of 5-oxoproline into glutamate which is an important neurotransmitter. This disorder is characterized by a large accumulation of 5-oxoproline in the urine. Symptoms of the disorder include enterocolitis, mental retardation, kidney stone formation, and hypoglycemia. 5-Oxoprolinase deficiency has been reported in approximately 8 people.

PW122045

Pw122045 View Pathway
disease

5-Oxoprolinase Deficiency

Rattus norvegicus
5-Oxoprolinase deficiency, also called OPLAHD, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of glutathione metabolism caused by a defective 5-oxoprolinase. 5-Oxoprolinase catalyzes the conversion of 5-oxoproline into glutamate which is an important neurotransmitter. This disorder is characterized by a large accumulation of 5-oxoproline in the urine. Symptoms of the disorder include enterocolitis, mental retardation, kidney stone formation, and hypoglycemia. 5-Oxoprolinase deficiency has been reported in approximately 8 people.

PW121821

Pw121821 View Pathway
disease

5-Oxoprolinase Deficiency

Mus musculus
5-Oxoprolinase deficiency, also called OPLAHD, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of glutathione metabolism caused by a defective 5-oxoprolinase. 5-Oxoprolinase catalyzes the conversion of 5-oxoproline into glutamate which is an important neurotransmitter. This disorder is characterized by a large accumulation of 5-oxoproline in the urine. Symptoms of the disorder include enterocolitis, mental retardation, kidney stone formation, and hypoglycemia. 5-Oxoprolinase deficiency has been reported in approximately 8 people.

PW000476

Pw000476 View Pathway
disease

5-Oxoprolinase Deficiency

Homo sapiens
5-Oxoprolinase deficiency, also called OPLAHD, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder of glutathione metabolism caused by a defective 5-oxoprolinase. 5-Oxoprolinase catalyzes the conversion of 5-oxoproline into glutamate which is an important neurotransmitter. This disorder is characterized by a large accumulation of 5-oxoproline in the urine. Symptoms of the disorder include enterocolitis, mental retardation, kidney stone formation, and hypoglycemia. 5-Oxoprolinase deficiency has been reported in approximately 8 people.

PW147030

Pw147030 View Pathway
metabolic

5-Hydroxy-L-tryptophan Drug Metabolism Pathway

Homo sapiens

PW125952

Pw125952 View Pathway
metabolic

5-Deoxystrigol Biosynthesis

Cannabis sativa
5-Deoxystrigol Biosynthesis is a pathway that has not yet become fully elucidated. Beginning in the chloroplast and potentially finishing in the cytosol, the pathway follows the synthesis of 5-deoxystrigol from beta-carotene. 5-Deoxystrigol is a strigolactone, a plant hormone that stimulates the branching and growth of symbiotic arbuscular mycorrhizal fungi and inhibits plant shoot branching. Strigolactones share a common C19 structure composed of a tricyclic lactone (A, B, and C rings) connected to a second lactone (D ring) by an enol ether bridge. 5-deoxystrigol is the precursor of other beta-oriented C-ring strigolactones (strigol-configured strigolactones) (PMID: 25425668). First, beta-carotene isomerase catalyzes the conversion of beta-carotene into 9-cis-beta-carotene with the help of an iron cofactor. Second, 9-cis-beta-carotene 9',10'-cleavage dioxygenase converts 9-cis-beta-carotene and oxygen to 9-cis-10'-apo-beta-carotenal and beta-ionone with the help of an Fe2+ cofactor. Third, carlactone synthase converts 9-cis-10'-apo-beta-carotenal and oxygen to carlactone and (2E,4E,6E)-7-hydroxy-4-methylhepta-2,4,6-trienal with the help of an Fe2+ cofactor. The final two reactions are not completely understood and may occur in the cytosol. Cytochrome P450 monooxygenase is theorized to catalyze the fourth reaction whereby carlactone is conveted into carlactone carboxylate. It requires heme as a cofactor. This same enzyme could possibly also catalyze the fifth reaction in which 5-deoxystrigol is made.

PW012878

Pw012878 View Pathway
metabolic

5-Deoxystrigol Biosynthesis

Arabidopsis thaliana
5-Deoxystrigol Biosynthesis is a pathway that has not yet become fully elucidated. Beginning in the chloroplast and potentially finishing in the cytosol, the pathway follows the synthesis of 5-deoxystrigol from beta-carotene. 5-Deoxystrigol is a strigolactone, a plant hormone that stimulates the branching and growth of symbiotic arbuscular mycorrhizal fungi and inhibits plant shoot branching. Strigolactones share a common C19 structure composed of a tricyclic lactone (A, B, and C rings) connected to a second lactone (D ring) by an enol ether bridge. 5-deoxystrigol is the precursor of other beta-oriented C-ring strigolactones (strigol-configured strigolactones) (PMID: 25425668). First, beta-carotene isomerase catalyzes the conversion of beta-carotene into 9-cis-beta-carotene with the help of an iron cofactor. Second, 9-cis-beta-carotene 9',10'-cleavage dioxygenase converts 9-cis-beta-carotene and oxygen to 9-cis-10'-apo-beta-carotenal and beta-ionone with the help of an Fe2+ cofactor. Third, carlactone synthase converts 9-cis-10'-apo-beta-carotenal and oxygen to carlactone and (2E,4E,6E)-7-hydroxy-4-methylhepta-2,4,6-trienal with the help of an Fe2+ cofactor. The final two reactions are not completely understood and may occur in the cytosol. Cytochrome P450 monooxygenase is theorized to catalyze the fourth reaction whereby carlactone is conveted into carlactone carboxylate. It requires heme as a cofactor. This same enzyme could possibly also catalyze the fifth reaction in which 5-deoxystrigol is made.