1 Orotic aciduria 2 G6PD deficiency Pyrimidine metabolism Glutamine CO2 Aspartate Dihydrofolate DHF reductase 9 Maturity onset diabetes of the young 10 Von Gierke disease 13 Lactose intolerance 14 Galactokinase deficiency 19 MCAD deficiency 20 Maple syrup urine disease ADP Galactose Aldose reductase ATP Galactitol 4-epimerase Fructokinase 16 Aldolase B 17 Dihydroxyacetone phosphate (DHAP) Fructose metabolism Glyceraldehyde NAD+ NADH Acyl-CoA synthase Glycerol 1,3-bisphosphoglycerate ADP 18 Triglycerides ATP IMP AMP 4 ADA Guanosine HGPRT Inosine Guanine PRPP Hypoxanthine 3 Adenosine Xanthine oxidase Fatty acid metabolism Acyl-CoA Carnitine CAT I Carnitine CAT II Acyl-carnitine Cytosol APRT Xanthine oxidase NH3 + CO2+ Carbamoyl phosphate synthetase I Phosphoenolpyruvate carboxykinase Aspartate ATP Citrulline Argininosuccinate synthase Ornithine transcarbamylase 5 Carbamoyl phosphate Lactate Malate Malate Succinate dehydrogenase H2O NADH Phenylalanine Melanin Phenylalanine hydroxylase Mevalonate 6 DOPA BH4 BH2 Dihydrobiopterin reductase HMG-CoA reductase TCA cycle NADH + CO2 FAD NAD+ NADH + CO2 Methylmalonyl-CoA 6 DOPA decarboxylase BH2 Dihydrobiopterin reductase Dopamine Acetoacetate Isocitrate dehydrogenase β-hydroxybutyrate α-Ketoglutarate α-Ketoglutarate dehydrogenase Ketone metabolism Pyrimidines + lipids Propionyl-CoA Branched-chain α-ketoacid dehydrogenase complex 20 α-ketoacid PhenylethanolamineN-methyltransferase 5, 10-methylene Methylene THF tetrahydrofolate reductase (MTHFR) 5-methyl THF THF Methionine Methionine synthase S-adenosyl methionine (SAM) Homocysteine 21 Cystathionine synthase Cystathionine Cysteine Cystathionase S-adenosyl homocysteine ATP Norepinephrine Amino acid metabolism HMG-CoA Isocitrate NAD+ FADH2 Acetoacetyl-CoA Tyrosinase 8 Tyrosine BH4 Cholesterol Methylmalonyl-CoA mutase Homogentisate 6 Cholesterol synthesis Citrate synthase Succinyl-CoA Homogentisic acid 7 dioxygenase Acetyl-CoA Citrate NAD + GTP GDP Maleylacetoacetate Thiamine (B1) Riboflavin (B2) Niacin (B3) Pantothenic acid (B5) Pyridoxine (B6) Biotin (B7) Folate (B9) Cobalamin (B12) NADH Oxaloacetate Succinate Arginase Acyl-CoA dehydrogenase Acetyl-CoA Fumarate Argininosuccinate lyase 19 Pyruvate 12 dehydrogenase NAD+ Malate dehydogenase Arginine Urea NADH Acyl-carnitine Acyl-CoA FADH2 Acetyl-CoA carboxylase ATP Pyruvate Argininosuccinate Ornithine Urea cycle Pyruvate kinase Oxaloacetate NADH NAD+ Malonyl-CoA Phosphoenolpyruvate ADP 11 Lactate dehydrogenase Urine Fatty acid synthase GDP Gluconeogenesis Uric acid Purine salvage pathway GTP Mit. Matrix Fatty acids 2-phosphoglycerate PRPP Adenine Xanthine 17 Fructose intolerance 18 Systemic 1° carnitine deficiency Fructose-1,6-bisphosphate Ribose-5phosphate GMP 15 Classic galactosemia 16 Essential fructosuria ATP Purine metabolism dUMP DTMP UDP-glucose ADP Pi Fructose-1,6bisphosphatase Glyceraldehyde3-phosphate AMP GMP Thymidylate synthase phosphate Lactase 13 Fructose1-phosphate IMP CTP Glactose-1-phosphate uridyltransferase Galactose-1- 14 Galactokinase UDP-galactose Glucose Fructose Hexokinase 15 3-phosphoglycerate 11 Pyruvate kinase deficiency 12 Pyruvate dehydrogenase deficiency PFK-1 Ribonucleotide reductase THF ATP ADP PRPP UDP 5,10methylene THF Transketolase Glucose-1phosphate Sucrose PRPP amidotransferase UMP dUDP Phosphoglucomutase Glucose-6-phosphate Fructose6-phosphate Lactose Glucose Glucose-6- 10 phosphatase Glucose-6- 2 phosphate dehydrogenase PRPP synthetase UMP synthetase 1 Orotic acid Transketolase Hexokinase / glucokinase Galactose metabolism Glycogenolysis / glycogenesis Glucose ADP NADP + 9 6-phosphogluconate Ribose-5phosphate Dihydroorotate dehydrogenase NADPH NADP+ Ribulose-5phosphate Carbamoyl phosphate 6 Phenylketonuria 8 Albinism NADPH Carbamoyl phosphate synthetase II 5 Ornithine transcarbamylase deficiency 7 Alkaptonuria Glycolysis ATP 3 Lesch-Nyhan syndrome 4 Severe combined immunodeficiency (SCID) HMP shunt • Valine Methylation • Isoleucine 21 Homocystinuria Epinephrine Copyright © 2018 by Physeo All rights reserved. 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Homocysteine metabolism www.physeo.com 1 Orotic aciduria 2 G6PD deficiency BIOCHEMISTRY MAP Pyrimidine metabolism Glutamine CO2 Aspartate 10 Von Gierke disease 13 Lactose intolerance 14 Galactokinase deficiency 19 MCAD deficiency 20 Maple syrup urine disease 21 Homocystinuria ATP Fructose-1,6bisphosphatase Glyceraldehyde3-phosphate ADP Aldose reductase ATP Galactitol 4-epimerase Fructokinase 16 ADP Aldolase B 17 Dihydroxyacetone phosphate (DHAP) Fructose metabolism Glyceraldehyde NAD+ NADH Purine metabolism Acyl-CoA synthase Glycerol 1,3-bisphosphoglycerate ADP 18 Triglycerides ATP IMP AMP 4 ADA Guanosine HGPRT Inosine Guanine PRPP Hypoxanthine 3 Adenosine Xanthine oxidase Fatty acid metabolism Acyl-CoA Carnitine CAT I Carnitine CAT II Acyl-carnitine Cytosol APRT Xanthine oxidase NH3 + CO2+ Carbamoyl phosphate synthetase I Citrulline Carbamoyl phosphate Malate Malate Succinate dehydrogenase Arginine Urea cycle Urea H2O Mevalonate TCA cycle Melanin Phenylalanine Phenylalanine hydroxylase NAD+ NADH + CO2 6 DOPA BH4 BH2 Dihydrobiopterin reductase Methylmalonyl-CoA 6 DOPA decarboxylase BH2 Dihydrobiopterin reductase Dopamine Acetoacetate Isocitrate dehydrogenase β-hydroxybutyrate α-Ketoglutarate dehydrogenase Ketone metabolism Pyrimidines + lipids Propionyl-CoA Branched-chain α-ketoacid dehydrogenase complex 20 α-ketoacid PhenylethanolamineN-methyltransferase Epinephrine 5, 10-methylene Methylene THF tetrahydrofolate reductase (MTHFR) 5-methyl THF THF Methionine Methionine synthase S-adenosyl methionine (SAM) Homocysteine 21 Cystathionine synthase Cystathionine Cysteine Cystathionase S-adenosyl homocysteine ATP Norepinephrine Amino acid metabolism HMG-CoA α-Ketoglutarate Tyrosinase 8 Tyrosine BH4 HMG-CoA reductase Acetoacetyl-CoA Isocitrate NAD+ NADH + CO2 FAD Methylmalonyl-CoA mutase Homogentisic acid 7 dioxygenase 6 Cholesterol synthesis Cholesterol Citrate synthase Succinyl-CoA Homogentisate Acetyl-CoA Citrate FADH2 GTP GDP Maleylacetoacetate Thiamine (B1) Riboflavin (B2) Niacin (B3) Pantothenic acid (B5) Pyridoxine (B6) Biotin (B7) Folate (B9) Cobalamin (B12) NADH NAD+ Succinate Arginase Acyl-CoA dehydrogenase Acetyl-CoA Oxaloacetate Fumarate Argininosuccinate lyase 19 NADH Pyruvate 12 dehydrogenase NAD+ Malate dehydogenase Argininosuccinate Ornithine NADH Acyl-carnitine Acyl-CoA FADH2 Acetyl-CoA carboxylase ATP Pyruvate Lactate NADH NAD+ Argininosuccinate synthase Ornithine transcarbamylase 5 Pyruvate kinase Oxaloacetate Aspartate ATP Malonyl-CoA Phosphoenolpyruvate ADP 11 Phosphoenolpyruvate carboxykinase Lactate dehydrogenase Urine Fatty acid synthase GDP Gluconeogenesis Uric acid Purine salvage pathway GTP Mit. Matrix Fatty acids 2-phosphoglycerate PRPP Adenine Xanthine 17 Fructose intolerance 18 Systemic 1° carnitine deficiency UDP-glucose Lactase 13 Galactose 3-phosphoglycerate 15 Classic galactosemia 16 Essential fructosuria phosphate Fructose1-phosphate Ribose-5phosphate DTMP GMP 11 Pyruvate kinase deficiency 12 Pyruvate dehydrogenase deficiency PFK-1 AMP GMP Thymidylate synthase Glactose-1-phosphate uridyltransferase Galactose-1- 14 Galactokinase UDP-galactose Glucose Fructose Hexokinase 15 Pi ADP dUMP THF 9 Maturity onset diabetes of the young CTP Glucose-1phosphate Sucrose Fructose6-phosphate Fructose-1,6-bisphosphate IMP Ribonucleotide reductase Dihydrofolate DHF reductase Phosphoglucomutase Glucose-6-phosphate ATP Lactose Glucose Glucose-6- 10 phosphatase Glucose-6- 2 phosphate dehydrogenase Transketolase Galactose metabolism Glycogenolysis / glycogenesis PRPP amidotransferase UMP dUDP ADP Hexokinase / 9 glucokinase PRPP UDP 5,10methylene THF Transketolase NADP+ PRPP synthetase UMP synthetase 1 Orotic acid 6-phosphogluconate Ribose-5phosphate Dihydroorotate dehydrogenase NADPH NADP+ Ribulose-5phosphate Carbamoyl phosphate 6 Phenylketonuria 8 Albinism NADPH Carbamoyl phosphate synthetase II 5 Ornithine transcarbamylase deficiency 7 Alkaptonuria Glycolysis Glucose ATP 3 Lesch-Nyhan syndrome 4 Severe combined immunodeficiency (SCID) HMP shunt • Valine • Isoleucine Methylation Homocysteine metabolism MEDICAL COURSE AND STEP 1 REVIEW FIRST EDITION Accompanies online videos taught by Michael Christensen & Rhett Thomson physeo.com