PathWhiz

PathWhiz ID	Pathway	Meta Data
PW315845 View Pathway	metabolic Pyruvate Decarboxylation to Acetyl-CoA Citrobacter youngae ATCC 29220 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 23:42 Last Updated: October 04, 2024 at 23:42
PW353434 View Pathway	metabolic Pyruvate Decarboxylation to Acetyl-CoA Escherichia coli O157:H7 str. TW14359 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 21:59 Last Updated: November 08, 2024 at 21:59
PW353439 View Pathway	metabolic Pyruvate Decarboxylation to Acetyl-CoA Escherichia coli O55:H7 str. CB9615 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 22:00 Last Updated: November 08, 2024 at 22:00
PW353427 View Pathway	metabolic Pyruvate Decarboxylation to Acetyl-CoA Escherichia coli UMN026 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 21:57 Last Updated: November 08, 2024 at 21:57
PW000117 View Pathway	disease Pyruvate Dehydrogenase Complex Deficiency Homo sapiens Pyruvate dehydrogenase complex deficiency results from a mutation in the E1-alpha polypeptide gene (PDHA1). PDHA1 encodes the pyruvate dehydrogenase complex (PDC) a critical complex that converts pyruvate from glycolysis to acetyl CoA for the citric acid cycle. This conversion step links glycolysis and the citric acid cycle. A defect in this complex causes accumulation of lactate and pyruvate in the blood; lactate and pyruvic acid in the spinal fluid; and lactate in the urine. Symptoms include lactic and metabolic acidosis, motor retardation, dystonia, growth and mental retardation, and respiratory distress. 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
PW122012 View Pathway	disease Pyruvate Dehydrogenase Complex Deficiency Rattus norvegicus Pyruvate dehydrogenase complex deficiency results from a mutation in the E1-alpha polypeptide gene (PDHA1). PDHA1 encodes the pyruvate dehydrogenase complex (PDC) a critical complex that converts pyruvate from glycolysis to acetyl CoA for the citric acid cycle. This conversion step links glycolysis and the citric acid cycle. A defect in this complex causes accumulation of lactate and pyruvate in the blood; lactate and pyruvic acid in the spinal fluid; and lactate in the urine. Symptoms include lactic and metabolic acidosis, motor retardation, dystonia, growth and mental retardation, and respiratory distress. 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
PW127339 View Pathway	disease Pyruvate Dehydrogenase Complex Deficiency Homo sapiens Pyruvate dehydrogenase complex deficiency results from a mutation in the E1-alpha polypeptide gene (PDHA1). PDHA1 encodes the pyruvate dehydrogenase complex (PDC) a critical complex that converts pyruvate from glycolysis to acetyl CoA for the citric acid cycle. This conversion step links glycolysis and the citric acid cycle. A defect in this complex causes accumulation of lactate and pyruvate in the blood; lactate and pyruvic acid in the spinal fluid; and lactate in the urine. Symptoms include lactic and metabolic acidosis, motor retardation, dystonia, growth and mental retardation, and respiratory distress. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: December 13, 2022 at 10:36 Last Updated: December 13, 2022 at 10:36
PW121787 View Pathway	disease Pyruvate Dehydrogenase Complex Deficiency Mus musculus Pyruvate dehydrogenase complex deficiency results from a mutation in the E1-alpha polypeptide gene (PDHA1). PDHA1 encodes the pyruvate dehydrogenase complex (PDC) a critical complex that converts pyruvate from glycolysis to acetyl CoA for the citric acid cycle. This conversion step links glycolysis and the citric acid cycle. A defect in this complex causes accumulation of lactate and pyruvate in the blood; lactate and pyruvic acid in the spinal fluid; and lactate in the urine. Symptoms include lactic and metabolic acidosis, motor retardation, dystonia, growth and mental retardation, and respiratory distress. 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
PW127324 View Pathway	disease Pyruvate Dehydrogenase Deficiency (E2) Homo sapiens Pyruvate Dehydrogenase (PDH) Deficiency is an X linked disease where individuals have a reduced number of functioning PDH complexes ultimately affecting the mitochondria’s energy metabolism. In a healthy individual, PDH complex catalyzes the conversion of pyruvate to acetyl coenzyme A, therefore PDH deficiency can cause the accumulation of excess pyruvate and lactic acid. PDH deficiency presents itself in a variety of ways, however since the brain obtains most of it’s energy from aerobic oxidation of glucose, all PDH deficient individuals have some degree of neurological impairment. Other symptoms range from fatal lactic acidosis in the newborns, chronic neurodegenerative conditions, brain lesions, cerebral atrophy and much more. Due to the fatal nature of the disease many with this condition do not live past childhood, however there are some that survive to adolescents and adulthood. Treatments have tried to minimize systemic lactic acid accumulation by feeding patients high fat/low carbohydrate diets. However, this does not reverse neurological structural damage already present and therefore does little to influence the end results. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: Ray Kruger Created On: December 09, 2022 at 10:21 Last Updated: December 09, 2022 at 10:21
PW000527 View Pathway	disease Pyruvate Dehydrogenase Deficiency (E2) Homo sapiens Pyruvate Dehydrogenase (PDH) Deficiency is an X linked disease where individuals have a reduced number of functioning PDH complexes ultimately affecting the mitochondria’s energy metabolism. In a healthy individual, PDH complex catalyzes the conversion of pyruvate to acetyl coenzyme A, therefore PDH deficiency can cause the accumulation of excess pyruvate and lactic acid. PDH deficiency presents itself in a variety of ways, however since the brain obtains most of it’s energy from aerobic oxidation of glucose, all PDH deficient individuals have some degree of neurological impairment. Other symptoms range from fatal lactic acidosis in the newborns, chronic neurodegenerative conditions, brain lesions, cerebral atrophy and much more. Due to the fatal nature of the disease many with this condition do not live past childhood, however there are some that survive to adolescents and adulthood. Treatments have tried to minimize systemic lactic acid accumulation by feeding patients high fat/low carbohydrate diets. However, this does not reverse neurological structural damage already present and therefore does little to influence the end results. BioPAX SVG SBGN SBML PWML All files (.zip)	Creator: WishartLab Created On: August 29, 2013 at 10:39 Last Updated: August 29, 2013 at 10:39