PathWhiz

PathWhiz ID	Pathway	Meta Data
PW122311 View Pathway	metabolic Beta-oxidation Homo sapiens BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Helen Broughton Created On: November 27, 2018 at 06:23 Last Updated: November 27, 2018 at 06:23
PW000187 View Pathway	disease beta-Ureidopropionase Deficiency Homo sapiens Beta-ureidopropionase deficiency (Beta Alanine-Synthase Deficiency, UPB1, BUP1) is an autosomal recessive disease caused by mutations in the UPB1 gene which codes for beta-ureidopropionase. A deficiency in this enzyme results in accumulation of N-carbamyl-beta-amino acids. Symptoms include hypotonia, dystonic movements, scoliosis, microcephaly, and severe developmental delay. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: WishartLab Created On: August 19, 2013 at 12:05 Last Updated: August 19, 2013 at 12:05
PW121709 View Pathway	disease beta-Ureidopropionase Deficiency Mus musculus Beta-ureidopropionase deficiency (Beta Alanine-Synthase Deficiency, UPB1, BUP1) is an autosomal recessive disease caused by mutations in the UPB1 gene which codes for beta-ureidopropionase. A deficiency in this enzyme results in accumulation of N-carbamyl-beta-amino acids. Symptoms include hypotonia, dystonic movements, scoliosis, microcephaly, and severe developmental delay. 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
PW127283 View Pathway	disease beta-Ureidopropionase Deficiency Homo sapiens Beta-ureidopropionase deficiency (Beta Alanine-Synthase Deficiency, UPB1, BUP1) is an autosomal recessive disease caused by mutations in the UPB1 gene which codes for beta-ureidopropionase. A deficiency in this enzyme results in accumulation of N-carbamyl-beta-amino acids. Symptoms include hypotonia, dystonic movements, scoliosis, microcephaly, and severe developmental delay. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: November 29, 2022 at 14:00 Last Updated: November 29, 2022 at 14:00
PW121934 View Pathway	disease beta-Ureidopropionase Deficiency Rattus norvegicus Beta-ureidopropionase deficiency (Beta Alanine-Synthase Deficiency, UPB1, BUP1) is an autosomal recessive disease caused by mutations in the UPB1 gene which codes for beta-ureidopropionase. A deficiency in this enzyme results in accumulation of N-carbamyl-beta-amino acids. Symptoms include hypotonia, dystonic movements, scoliosis, microcephaly, and severe developmental delay. 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
PW061694 View Pathway	drug action Betahistine H1-Antihistamine Action Homo sapiens Betahistine is a first-generation H1-antihistamine. H1-antihistamines interfere with the agonist action of histamine at the H1 receptor and are administered to attenuate inflammatory process in order to treat conditions such as allergic rhinitis, allergic conjunctivitis, and urticaria. Reducing the activity of the NF-κB immune response transcription factor through the phospholipase C and the phosphatidylinositol (PIP2) signalling pathways also decreases antigen presentation and the expression of pro-inflammatory cytokines, cell adhesion molecules, and chemotactic factors. Furthermore, lowering calcium ion concentration leads to increased mast cell stability which reduces further histamine release. First-generation antihistamines readily cross the blood-brain barrier and cause sedation and other adverse central nervous system (CNS) effects (e.g. nervousness and insomnia). Second-generation antihistamines are more selective for H1-receptors of the peripheral nervous system (PNS) and do not cross the blood-brain barrier. Consequently, these newer drugs elicit fewer adverse drug reactions. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Carin Li Created On: September 20, 2017 at 11:22 Last Updated: September 20, 2017 at 11:22
PW176323 View Pathway	metabolic Betahistine Predicted Metabolism Pathway Homo sapiens Metabolites of sildenafil are predicted with biotransformer. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Omolola Created On: December 07, 2023 at 15:05 Last Updated: December 07, 2023 at 15:05
PW126232 View Pathway	metabolic Betaine Drug Metabolism Homo sapiens Betaine is obtained from foods such as wheat, shellfish, spinach, and beets. In the intestine, betaine is metabolized to trimethylamine (TMA) by the gut microbiota (Peptoclostridium acidaminophilum). The toxicity of betaine is due to the formation of TMA and its metabolism to Trimethylamine N-oxide (TMAO) in the liver. TMA is created from betaine via the enzyme betaine reductase in the intestinal microbe, then TMA then enters the bloodstream and is transported to the liver where dimethylaniline monooxygenase [N-oxide-forming] 3 converts TMA to TMAO. TMAO has negative effects on organs such as the heart, kidney and vascular system by contributing to cardiovascular disease, atherosclerosis, endothelial dysfunction and kidney disease. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Karxena Harford Created On: September 14, 2021 at 23:25 Last Updated: September 14, 2021 at 23:25
PW145771 View Pathway	drug action Betaine Drug Metabolism Action Pathway Homo sapiens BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: October 07, 2023 at 16:36 Last Updated: October 07, 2023 at 16:36
PW088201 View Pathway	metabolic Betaine Metabolism Bos taurus Betaine (or trimethylglycine) is similar to choline (trimethylaminoethanol) but differs in choline's terminal carboxylic acid group trimethylglycine is reduced to a hydroxyl group. Betaine is obtained from diet as betaine or compounds containing choline in foods such as whole grains, beets and spinach. Betaine can also be synthesized from choline in the liver and kidney. First, choline is oxidized to betaine aldehyde by mitochondrial choline oxidase (choline dehydrogenase). Then, betaine aldehyde dehydrogenase oxidizes betaine aldehyde to betaine in the mitochondria or cytoplasm. In the liver, betaine functions as a methyl donor similar to choline, folic acid, S-adenosyl methionine and vitamin B12. Methyl donors are important for liver function, cellular replication and detoxification reactions. Betaine is also involved in the production of carnitine to protect from kidney damage and functions as an osmoprotectant in the inner medulla. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ana Marcu Created On: August 10, 2018 at 11:06 Last Updated: August 10, 2018 at 11:06