Double bond on the side chain is reduced
Double bond is moved from C8-9 to C5-C6
Methyl groups of C14 and 4 are removed
Lanosterol Cyclase
Squaline Epoxidase
Mevalonate Kinase
HMG-CoA Reductase
HMG-CoA Synthase
Thiolase
Thiolase
3-Hydroxyacyl CoA Dehydrogenase
(2,3-)Enoyl CoA Hydratase
Acyl CoA Dehydrogenase
CTP Synthase
kinase
kinase
translocase
kinase
Bilirubin glucuronyltransferase
Biliverdin Reductase
Heme Oxygenase
Ferrocheletase
Uroporphyrinogen Decarboxylase
Uroporphyrinogen III Synthase
phenylethanolamine N methyltransferase
Dopamine Hydroxylase (vitamin C, Cu2+)
DOPA Decarboxylase (PLP)
Tyrosinase
4-Malylacetoacetate Isomerase
Homogentisate oxidase
p-Hydroxyphenylpyruvate Hydroxylase
Tyrosine Hydroxilase (B4H, Fe2+)
Tyrosinase
Tyrosine Aminotransferase
Phenylalanine Hydroxylase
Glutaminase
galactokinase
Lactase
fructokinase
Acetaldehyde Dehydrogenase
Alcohol Dehydrogenaase
6PG Dehydrogenase
Decarboxylation
Glycosyltransferase
UDP-glucose dehydrogenase
UDP-Glucose pyrophosphorylase
Glycogen phosphorylase (active form: phosphorylated
Creatine Kinase (muscle, liver, brain)
Guanidoacetate methyltransferase (liver)
Arginine-Glycine transaminidinae (Kidneys)
Arginase
Argininosuccinate Lyase
Argininosuccinate Synthetase I
Carbamoyl Phosphate Synthase I ( CPS I > Mitochondrium)
acyltransferase
phosphatase
Acyltransferase
Triglyceride Lipase
Diglyceride Lipase
Monoglyceride lipase
glycerol-3-phosphate dehydrogenase
translocase
Acyltransferase
Carnitine-Acylcarnitine Transferase 1
Thiokinase (or Acyl CoA Synthetase) (Cytosol)
Acetyl CoA Carboxylase
Citrate Lyase
Citrate Shuttle -> Cytosol
aconitase
isocitrate dehydrogenase
δ-AminoLevulinic Acid Synthase (PLP needed)
α-ketoglutarate dehydrogenase
S-tiokinase
Succinate Dehydrogenase
Fumarase
Malate Dehydrogenase
Thiolase
Thiphorase or CoA Transferase (not available in hepatocytes)
3-Hydroxybutarate Dehydrogenase
3-Hydroxybutarate Dehydrogenase
3H3MG CoA Lyase
Mitochondrial 3H3MGCoA Synthatase
Thiolase
Pyruvate Carboxylase (Mitochondria)
Pyruvate Decarboxylase (Reduced)
Pyruvate Dehydrogenase (Decarboxylation) (activated by desphosphorylation) (In the Mirochondria)
LDH
Pyruvate Kinase (activated by dephosphorylation)
Enolase
phosphoglycerate mutase
Biphosphoglyceratemutase
Phosphoglycerate kinase
Glyceradehyde phsophate dehydrogenase
Aldolase
Phosphofructokinase-1
Glucose 6-phosphate Dehydrogenase (Rate limiting step)
phosphoglucose isomerase
Hexokinase (or glucokinase)
Orotate phosphoribosyl tranferase
Glucosidase (only 10% of broken down Glycogen)
PRPP synthetase
kinase
phosphoglucomutase
Kinase
Coproporphyrinogen Oxidase
10 reactions total
Aspartate transcarbamoylase
PFK-2
Fumarylacetoacetate hydroxylase
Glucose-6-Phosphatase (present in the liver, kidney cortex)
Porphobilinogen Synthase
Citrate Synthetase
OMP decarboxylase
Adenylosuccinate Synthase
UDP-Gal Epimerase
Carnitine-Acyl-Carnitine Transferase 2
Phospho-enol Pyruvate-carboxy kinase
Xanthine oxidase
Dihydroorotase
Ornithine Transcarbamylase
Fumarase
Lyase
triose phosphate isomerase
IMP Dehydrogenase
transferase
Xanthine oxidase
6-Phosphogluconolactone Hydrolase
Glutamine Synthetase
Fructose 1,6 phosphatase
Acetyl-CoA Synthetase
Protoporphyrinogen Oxidase
Glycerol Kinase (phosphorylates glycerol)
Glycogen synthase (active: dephosphorylated)
Porphobilinogen Deaminase
AMP kinase
Gal-1-P Uridyltransferase
Malate dehydrogenase
triokinase
aldose
GMP kinase
PDH
Carbamoylphosphate synthase II (CPS II - cytosol)
Biphosphoglyceratephosphatase
Adolase B
Guanine deaminase
Dihydroorotate dehydrogenase

Glucose

c2

Glucose 6-phosphate

c1

Fructose 6-phosphate

Fructose 1,6-biphosphate

c1

Glyceraldehyde 3-phosphate x2

1,3-biphosphoglycerate x2

3-phosphoglycerate x2

2-phosphoglycerate x2

phospho-enol-pyruvate x2

Pyruvate x2

c1

Lactate

Cori's Cycle

Acetyl CoA

c4

Acetoacetyl CoA

3-Hydroxy-methylglutaryl CoA

Acetoacetate

3-Hydroxybutyrate

Acetoacetate

Acetoacetyl CoA

Acetyl CoA x2

Acetone

CO2

Alanine

c1

Ethanol (not in humans)

Oxaloacetate

c1

Malate

Fumarate

Succinate

Succinyl CoA

c1

α-ketoglutarate

c1

Isocitrate

Citrate

c1

Citrate

Ocaloacetate

Acteyl-CoA

Malonyl CoA

c2

Fatty Acid Synthase

c1

Condensation

Reduction

Dehydration

Reduction

Palmitic Acid C16

c2

Fatty Acid

Fatty Acid-Acyl CoA

Acylcarnitine

c1

Acylcarnitine

CoA

Phosphatidic Acid

Diglyceride

Diacylglyceride (DAG)-CDP
(Lecithin or sphingosine)

c1

Catabolism

Phospholipase A2
(mammals)

c1

Phospholipase A1
(mammals)

Phospholipase D
(plants)

Phospholipase C
(mammals and bacteria)

c1

Phosphate

2x COASH

CoA

Glycolipids

c1

NADPH

NADPH

Glutamate
Glutamine
Arginine
Histidine
Proline

δ-AminoLevulinic Acid

c1

Porphobilinogen

c2

CoA and Glycine (?)

2,3-Diphosphoglycerate

c1

6-Phosphogluconolactone

c1

Fructose 2,3 biphosphate

Dihydoxyacetone phosphate

Glycerol-3-phosphate

2-Monoacylglyecerol

Glycerol

1,2 Diacylglycerol

Triacylglycerol

Diacylglycerol

Phosphatidic Acid

Glycerol-3-Phosphate

α-ketoglutarate

glutamate

NH3

Carbamoyl Phosphate

c3

Citrulline

Argininosuccinate

Arginine

Ornithine

Urea

Guanidoacetate

Creatine

Creatine phosphate

P

Creatinine

Urine

Peeeeeeeeeee

H2O

Fumarate

NADH

NAD+

FAD2+

FADH2

GDP

GTP

NADH

NAD+

NAD+

NADH

Krebs Cycle

Glycogen

c4

Glucose-1-phosphate

UDP Glucose

UDP-Glucuronate

c1

Glycoaminoglycans

D Glucoronic Acid

L-Xylulose

UDP Galactose

NADP+

NADPH

c4

Acetyl CoA

Acetyl CoA

NADH

NAD+

Succinyl CoA

Succinate

CoA

Muscle Tissue

Liver Tissue

Pentose Pathway Shunt

NADP+

6-phosphogluconate

Ribulose 5-phosphate

Xylulose-5-phosphate

NADPH

c4

NADH

NAD+

Acetyl CoA

Ethanol

Acetaldehyde

acetate

NAD+

NAD+

NADH

NADH

ATP

AMP

CoA

CO2

Simplified Glucuronic Acid Shit

ATP

ADP

ATP,
NADH

CO2

CO2

ATP,
Succinyl CoA,
NADH

Ketogenesis

c1

Alcohol Metabolism

ATP

ADP

UTP

PPi

Fructose

c1

Fructose-1-phosphate

Glyceraldehyde

Lactose

Galactose

Galactose-1-phosphate

ATP

ADP

H2O

Glucose

CO2

H2O

CO2

UREA CYCLE

ATP

ADP

ATP

ADP

ATP

ADP

NAD+

NADH

Glutamate

α-ketoglutarate

Glutamine

NH3

NAD+

NADH

NADH

NAD+

Glutamate Dehdrogenase

c1

NH3

ATP?

ADP

H2O

Alanine Aminotransferase ALAT = GPT

c1

α-ketoglutarate

Glutarate

Aspartate

Oxaloacetate

Aspartate Aminotransferase ASAT = GOT

Glucogenic:
-Glycine
-Alanine (major)
-Serine
-Cysteine
-Threonine
-Tryptophan

Isoleucine
Methionine
Threonine
Valine

Phenylalanine

Tyrosine

4-Hydroxyphenylpyruvate

Homogentisate

4-Maleylacetoacetate

4-Fumarylacetoacetate

Acetoacetate

Dihydroxy Phenylalanine (DOPA)

DOPA-Quinone

Melanin

L-DOPA

Dopamine

Norepinephrine

Epinephrine

c1

T3

c1

T4

c1

Deficiency: Phenyketonuria

c4

Deficiency: Tyrosinemia II (Occulucutaneous)

c1

Deficiency: Alkaptonuria

c2

Deficiency: Tyrosinemia III (Neonatal)

c2

Deficiency: Tyrosinemia I (Hepatorenal)

c2

NADPH

NADP+

Deficiency: Albinism

O2

O2

Aspaargine

Isoleucine
Leucine
Tryptophan

Leucine
Lycine
Phenylalanine
tryptophan
Tyrosine

Ketogenic Amino Acids

Glycine

Ornithine

ATP + SAM

S-Adenosylhomocysteine +
ADP

ADP + P

ATP

CREATINE

Catecholamine Synthesis

Melanin Synthesis

δ-AminoLevulinic Acid

Porphobilinogen

Hydroxymethylbilane

Uroporphyrinogen III

Vitamin B12

Chlorophyl

Protoporphyrin IX

Heme

Heme

Biliverdin

Unconjugated Bilirubin

Albumin-Bilirubin Complex

c1

Unconjugated Bilirubin

Conjugated Bilirubin (Diglucuronide)

Urobilinogen

Stercobilin

Urobilinogen

Porphobilinogen

Porphobilinogen

Glycine

In Macrophages

In the Blood

In the Liver

In the intestines

Kidneys

O2

NADPH

NADP

NADPH

NADP

2 X UDP-Glucuronic Acid

2 x UDP

Stool

Heme Synthesis

Heme Degradation

Fe3+, CO

Deficiency

c3

Deficiency

c2

Deficiency

c3

Deficiency

c3

Deficiency

c2

10X more activity

Glycolysis

c2

GLUCONEOGENISIS

Rapoport-Luebering Cycle

Deficiency

c2

Deficiency

c3

ATP

ADP

Fatty Acid-Acyl CoA

Glycerol

α-glycerophosphate

Choline or Ethanolamine

CDP-Choline or CDP-Ethanolamine

Malate

Oxaloacetate

x2 ATP

2x ADP

H2O

CoA

Carnitine

Cytosol

FAD

FADH2

H2O

NAD

NADH

Acetyl CoA

Acetyl CoA

Rate Limiting Step

c4

ATP

ADP

Farnesyl Diphosphate

H2O

H2O

Ribose-5-phosphate

Fructose-6-phosphate

Glycerinaldehyde-3-phosphate

Phosphoribosyl-pyrophosphate PRPP

c1

PURINE SYNTHESIS (Adenine, Guanine)

c3

IMP (inosine monophosphate) = parent of purines

Gutamine

c2

Glycine

Aspartate

AMP (Adenosine-monophosphate)

c1

GMP (Guanosine monophosphate)

c1

Adenylosuccinate

Xanthosine monophosphate

CO2

GDP

ADP

GTP

ATP

- ATP

-ATP

-ATP

-ATP

Guanine

Adenosine

Hypoxanthine

Inosine

Xanthine

Uric acid

c2

PURINE DEGRADATION

Formyl FH

urine

c1

HGPRT

c2

HGPRT

c1

Adenin

APRT

c1

PURINE SALVAGE PATHWAY (Wiederverwertung)

IMP, AMP, GMP do not need de novo synthesis at all (only salvage pathway)

Glutamine

Glutamate

HCO3-

2 ATP

2 ADP

H2O

Carbamoylphosphate

Carbamoyl aspartate

c1

Dihydroorotate (DHO)

Orotate

OMP "parent of pyrimidines"

UMP

UDP

UTP

CTP

CTP

c1

CDP

dCDP

c1

dCTP

dCMP

dUDP

dUMP

dTMP

c1

dTDP

dTTP

PYRIMIDINE SYNTHESIS

c4

ATP

ADP

ATP

ADP

ATP

ADP

Glutamine

Glutamate

P

Uridine
Cytidine

UMP
CMP

Deoxythymidine

dTMP

Deoxyctidine

dCMP

Uracil
Thymine
Orotic Acid

c1

UMP
dTMP
OMP

ATP

ADP

ATP

ADP

ATP

ADP

PPRP

PP

Salvage Pathway

Fatty Acid Syntehsis

Phospholipid Synthesis (in microsomes)

Beta Oxidation

Cholesterol Synthesis

Serine too?

Methionine

Deficiency

c3

Deficiency

c2

Deficiency

c1

Triglyceride Synthesis

Lipolysis

c1

HCO3

Activation

Carnitine

c1

Lysin
Methionine

Fatty Acid-Acyl CoA

Trans-2-Enoyl CoA

3-Hydroxyacyl CoA

3-Ketoacyl CoA

C14 Acyl-CoA

Acetyl CoA

Acetoacertyl CoA

3-Hydroxy-3-Methylglutaryl CoA

Mevaolnate

Mevalonate-5-Phosphate

5 Pyrophosphomevalonate

Isopentylyl Pyrophosphate

Farnesyl Diphosphate

Squalene

Squaline 2,3 Epoxide

Lanosterol

Zymosterol

Desmosterol

Cholesterol

c1

Mitochondria

Cytosol

Deficiency

c1

Deficiency

c1

Deficiency

c1

D-Xylulose 5-Phosphate
(to pentose shunt)

Deficiency

c1

Deficiency

c1

Deficiency

c2

Pyruvate

Lactate !!

PRPP amidotransferase

Fe2+

Aspartate

Pi

NAD

NADH

Aspartate

Pi

PRPP

In the Liver

Outside the Liver

Proprionate

precursors: lysine + methionine