Physeo Biochemistry Map [1 ed.]

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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. No part of this publication may be reproduced, distributed, or transmitted in any form or by any means, including photocopying, recording, or other electronic or mechanical methods, without the prior written permission of Physeo, except in the case of personal study purposes.

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

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