PathWhiz

PathWhiz ID	Pathway	Meta Data
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. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: WishartLab Created On: August 29, 2013 at 10:38 Last Updated: August 29, 2013 at 10:38
PW147030 View Pathway	metabolic 5-Hydroxy-L-tryptophan Drug Metabolism Pathway Homo sapiens BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: October 10, 2023 at 13:41 Last Updated: October 10, 2023 at 13:41
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. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Eponine Oler Created On: May 04, 2021 at 12:43 Last Updated: May 04, 2021 at 12:43
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. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Carin Li Created On: February 08, 2017 at 12:54 Last Updated: February 08, 2017 at 12:54
PW147006 View Pathway	metabolic 5-Aminolevulinic acid Drug Metabolism Pathway Homo sapiens BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: October 10, 2023 at 13:38 Last Updated: October 10, 2023 at 13:38
PW246846 View Pathway	metabolic 4-Hydroxyphthalate degradation Comamonas testosteroni Bacteria such as Comamonas testosteroni plays a crucial role in degradation of 4-hydroxyphthalate, an environmental pollutant. 4-hydroxyphthalate is degraded by Comamonas testosterone, yielding precursors used in the benzoate degradation pathway to yield carbon and energy, which is vital for the bacteria's growth and survival. Although the precise mechanisms of 4-hydroxyphthalate uptake, potentially facilitated by MFS transporters, are not fully understood, the subsequent enzymatic breakdown within the cell yields 4-Carboxy-2-hydroxymuconate semialdehyde, an essential intermediate for the benzoate degradation pathway. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Julia Wakoli Created On: May 13, 2024 at 15:39 Last Updated: May 13, 2024 at 15:39
PW127351 View Pathway	disease 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency Homo sapiens 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency (SSADH; Gamma-hydroxybutyric acidemia) inhibits the formation of succinate from GABA. This deficiency results in urinary excretion of 4-hydroxybutyric acid. In vivo proton MR also indicates elevated GABA levels as compared with an age-matched control. Symptoms include ataxia, chorea or athetosis, motor retardation, seizures, macrocephaly and delayed or abnormal speech development. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: December 14, 2022 at 13:57 Last Updated: December 14, 2022 at 13:57
PW121696 View Pathway	disease 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency Mus musculus 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency (SSADH; Gamma-hydroxybutyric acidemia) inhibits the formation of succinate from GABA. This deficiency results in urinary excretion of 4-hydroxybutyric acid. In vivo proton MR also indicates elevated GABA levels as compared with an age-matched control. Symptoms include ataxia, chorea or athetosis, motor retardation, seizures, macrocephaly and delayed or abnormal speech development. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:49 Last Updated: September 10, 2018 at 15:49
PW000070 View Pathway	disease 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency Homo sapiens 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency (SSADH; Gamma-hydroxybutyric acidemia) inhibits the formation of succinate from GABA. This deficiency results in urinary excretion of 4-hydroxybutyric acid. In vivo proton MR also indicates elevated GABA levels as compared with an age-matched control. Symptoms include ataxia, chorea or athetosis, motor retardation, seizures, macrocephaly and delayed or abnormal speech development. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: WishartLab Created On: August 01, 2013 at 15:52 Last Updated: August 01, 2013 at 15:52
PW121922 View Pathway	disease 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency Rattus norvegicus 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency (SSADH; Gamma-hydroxybutyric acidemia) inhibits the formation of succinate from GABA. This deficiency results in urinary excretion of 4-hydroxybutyric acid. In vivo proton MR also indicates elevated GABA levels as compared with an age-matched control. Symptoms include ataxia, chorea or athetosis, motor retardation, seizures, macrocephaly and delayed or abnormal speech development. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: September 10, 2018 at 15:51 Last Updated: September 10, 2018 at 15:51