PathWhiz

PathWhiz ID	Pathway	Meta Data
PW000058 View Pathway	disease Pyruvate Carboxylase Deficiency Homo sapiens Pyruvate carboxylase deficiency is caused by mutation in the pyruvate carboxylase gene. Serine—pyruvate aminotransferase catalyzes the reaction of serine and pyruvate to produce 3-hydroxypyruvate and L-alanine, as well as the reaction from L-alanine and glyodxylate to pyruvate and glycine. A defect in this results in accumulation of ammonia, glucose and pyruvate in blood; proline, lysine, citrulline, and alanine in plasma; and 2-oxoglutaric acid, fumaric acid, ketone bodies and succinate in urine. Symptoms include ataxia, lactic acidosis, mental retardation, metabolic acidosis, siezures, and dyspnea. 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
PW127317 View Pathway	disease Pyruvate Carboxylase Deficiency Homo sapiens Pyruvate carboxylase deficiency is caused by mutation in the pyruvate carboxylase gene. Serine—pyruvate aminotransferase catalyzes the reaction of serine and pyruvate to produce 3-hydroxypyruvate and L-alanine, as well as the reaction from L-alanine and glyodxylate to pyruvate and glycine. A defect in this results in accumulation of ammonia, glucose and pyruvate in blood; proline, lysine, citrulline, and alanine in plasma; and 2-oxoglutaric acid, fumaric acid, ketone bodies and succinate in urine. Symptoms include ataxia, lactic acidosis, mental retardation, metabolic acidosis, siezures, and dyspnea. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: December 07, 2022 at 14:45 Last Updated: December 07, 2022 at 14:45
PW121789 View Pathway	disease Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency) Mus musculus Pyruvate Decarboxylase E1 Component Deficiency is caused by a defect in the PDHA1 gene which codes for mitochondrial pyruvate dehydrogenase E1 component subunit alpha, somatic form. This is a homotetrameric enzyme that catalyses the decarboxylation of pyruvic acid to acetaldehyde and carbon dioxide. A defect in this enzyme results in accumulation of lacate and pyruvate. Symptoms and signs include severe lactic acidosis in the newborns that usually leading to death, hypotonic, lethargic, seizures, mental retardation and spasticity. 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
PW127338 View Pathway	disease Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency) Homo sapiens Pyruvate Decarboxylase E1 Component Deficiency is caused by a defect in the PDHA1 gene which codes for mitochondrial pyruvate dehydrogenase E1 component subunit alpha, somatic form. This is a homotetrameric enzyme that catalyses the decarboxylation of pyruvic acid to acetaldehyde and carbon dioxide. A defect in this enzyme results in accumulation of lacate and pyruvate. Symptoms and signs include severe lactic acidosis in the newborns that usually leading to death, hypotonic, lethargic, seizures, mental retardation and spasticity. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: December 13, 2022 at 10:07 Last Updated: December 13, 2022 at 10:07
PW122014 View Pathway	disease Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency) Rattus norvegicus Pyruvate Decarboxylase E1 Component Deficiency is caused by a defect in the PDHA1 gene which codes for mitochondrial pyruvate dehydrogenase E1 component subunit alpha, somatic form. This is a homotetrameric enzyme that catalyses the decarboxylation of pyruvic acid to acetaldehyde and carbon dioxide. A defect in this enzyme results in accumulation of lacate and pyruvate. Symptoms and signs include severe lactic acidosis in the newborns that usually leading to death, hypotonic, lethargic, seizures, mental retardation and spasticity. 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
PW000116 View Pathway	disease Pyruvate Decarboxylase E1 Component Deficiency (PDHE1 Deficiency) Homo sapiens Pyruvate Decarboxylase E1 Component Deficiency is caused by a defect in the PDHA1 gene which codes for mitochondrial pyruvate dehydrogenase E1 component subunit alpha, somatic form. This is a homotetrameric enzyme that catalyses the decarboxylation of pyruvic acid to acetaldehyde and carbon dioxide. A defect in this enzyme results in accumulation of lacate and pyruvate. Symptoms and signs include severe lactic acidosis in the newborns that usually leading to death, hypotonic, lethargic, seizures, mental retardation and spasticity. 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
PW353419 View Pathway	metabolic Pyruvate Decarboxylation to Acetyl-CoA Escherichia coli APEC O1 The multi-enzyme complex catalyze three reactions that constitute a cycle, which is an essential source of acetyl-CoA. Pyruvate dehydrogenase E1 and dihydrolipoyllysine-residue acetyltransferase catalyzes pyruvic acid to acetyl-CoA and CO2. Acetyl-CoA is required for the TCA cycle. Lipoamide dehydrogenase reduces NAD+ to NADH. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Julia Wakoli Created On: November 08, 2024 at 21:55 Last Updated: November 08, 2024 at 21:55
PW353421 View Pathway	metabolic Pyruvate Decarboxylation to Acetyl-CoA Escherichia coli HS The multi-enzyme complex catalyze three reactions that constitute a cycle, which is an essential source of acetyl-CoA. Pyruvate dehydrogenase E1 and dihydrolipoyllysine-residue acetyltransferase catalyzes pyruvic acid to acetyl-CoA and CO2. Acetyl-CoA is required for the TCA cycle. Lipoamide dehydrogenase reduces NAD+ to NADH. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Julia Wakoli Created On: November 08, 2024 at 21:56 Last Updated: November 08, 2024 at 21:56
PW315875 View Pathway	metabolic Pyruvate Decarboxylation to Acetyl-CoA Edwardsiella tarda ATCC 23685 The multi-enzyme complex catalyze three reactions that constitute a cycle, which is an essential source of acetyl-CoA. Pyruvate dehydrogenase E1 and dihydrolipoyllysine-residue acetyltransferase catalyzes pyruvic acid to acetyl-CoA and CO2. Acetyl-CoA is required for the TCA cycle. Lipoamide dehydrogenase reduces NAD+ to NADH. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Julia Wakoli Created On: October 04, 2024 at 23:57 Last Updated: October 04, 2024 at 23:57
PW315868 View Pathway	metabolic Pyruvate Decarboxylation to Acetyl-CoA Trabulsiella guamensis ATCC 49490 The multi-enzyme complex catalyze three reactions that constitute a cycle, which is an essential source of acetyl-CoA. Pyruvate dehydrogenase E1 and dihydrolipoyllysine-residue acetyltransferase catalyzes pyruvic acid to acetyl-CoA and CO2. Acetyl-CoA is required for the TCA cycle. Lipoamide dehydrogenase reduces NAD+ to NADH. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Julia Wakoli Created On: October 04, 2024 at 23:54 Last Updated: October 04, 2024 at 23:54