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Biochem Map

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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
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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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