Enzyme

Involved in

From

To

Activators

Inhibitors

constitutive

G-6-P

Glycolysis

Glucose

G-6-P

Hexokinase

Mannose
metabolism

Mannose

Mannose-6-P

Glucokinase

Glycolysis

Glucose

G-6-P

insulin

F-6-P

PFK-1

Glycolysis

F-6-P

Glyceraldehyde-3-P

Fructose-2,6-P,
AMP, insulin

Citrate, ATP

Pyruvate kinase

Glycolysis

PEP

Pyruvate

Fructose-1,6-P
(feed-forward),
insulin

Alanine, ATP

Lactate dehydrogenase (LDH)

Anaerobic
glycolysis

Pyruvate

Lactate

Cofactors

NADH
Pyruvate, CoA,
NAD, PDH
phosphatase,
Mg2+, Ca2+,
dichloroacetate

Acetyl-CoA,
NADH, PDH
kinase

Thiamine pyrophosphate
(TPP)
Lipoic acid
CoA (B5)
FAD (B2)
NAD (B3)

Pyruvate dehydrogenase (PDH)

Aerobic
glycolysis

Pyruvate

Citrate synthase

TCA

Acetyl-CoA, Oxaloacetate Citrate

Acetyl-CoA,
Oxaloacetate

Citrate,
Succinyl-CoA

Isocitrate dehydrogenase

TCA

Isocitrate

ADP, Ca2+

NADH

ADP, Ca2+

Thiamine pyrophosphate
(TPP)
NADH, Succinyl- Lipoic acid
CoA, CoA, GTP CoA (B5)
FAD (B2)
NAD (B3)

Acetyl-CoA

-Ketoglutarate

-Ketoglutarate dehydrogenase

TCA

-Ketoglutarate

Succinyl-CoA

Fructokinase

Fructose
metabolism

Fructose

Fructose-1-P

Aldolase B

Fructose
metabolism

Fructose-1-P

DHAP, Glyceraldehyde

Glyceraldehyde

Glyceraldehyde-3-P

Galactose

Galactose-1-P

Glyceraldehyde kinase
Galactokinase

Fructose
metabolism
Galactose
metabolism

transferase,
epimerase

NAD

G-6-P dehydrogenase
6-Pgluconate dehydrogenase

PPP

G-6-P

Pentose phosphate,
NADPH

insulin

NADPH

Enzyme

Involved in

From

To

Activators

Inhibitors

Transketolase

PPP

Transaldolase

PPP

-Glutamyl transpeptidase

Brings aa into
cell

Amino acid

Amino acid combines w/

glutathione

Glutamine synthetase

Dietary Proteins

Glutamate (1N)

Glutamine (2N)

Glutaminase

Dietary Proteins

Glutamine (2N)

Glutamate (1N)

Alanine aminotransferase (ALT)

Aspartate aminotrasferase (AST)

Dietary Proteins:
Transam.,
Decarbox.

B1

Dietary Proteins

Glutamate (reversible)

-Ketoglutarate

CPS-1

Urea Cycle

NH4+, CO2

Carbamoyl phosphate

Dietary Proteins

-cotransported w/ Na+
-use ATP to synth.
glutathione

-amino acid <---- ----> -keto acid

Alanine <---- ----> Pyruvate
Glutamate <---- ----> -Ketoglutarate
Aspartate <---- ----> Oxaloacetate
Histidine <---- ----> Histamine

Glutamate dehydrogenase (GDH)

Branched-chain -ketoacid
dehydrogenase

Cofactors

Alpha-ketoacids

Acyl-CoA derivatives

Pyridoxal phosphate
(PLP - B6)

ADP, GDP (rxn goes ATP, GTP (rxn

right)
goes left)
presence of amino
acids

NAD, NADP
ATP
Thiamine pyrophosphate
(TPP)
Lipoic acid
CoA (B5)
FAD (B2)
NAD (B3)

Phenylalanine hydroxylase
Phenylalanine

Dihydobiopterin
reductase/synthetase
TH4
S-adenosylmethionine (SAM)

One Carbon
Metabolism

Tyrosine
B6, B12, folate

Glutamate decarboxylase

Synth of GABA

Glutamate

GABA

BH4, PLP

Synth of
Serotonin

Tryptophan

Serotonin

Enzyme

Involved in

From

To

Glucose-1-P

UDP-glucose

UDP-glucose

UDP-glucose pytophosphorylase
Glycogen synthase
Glycogen phosphorylase

Glycogen
Metabolism
Glycogen
Metabolism
Glycogen
Metabolism

B6, B12, folate

Activators

Inhibitors

Glycogen

insulin, G-6-P

protein kinase A

Glycogen

Glucose-1-P

protein kinase A,
AMP, Ca2+

insulin,
G-6-P

Glucose-1-P

Glucose-6-P

Phosphoglucomutase

Glycogen
Metabolism

Glucose-6-phosphatase

Glycogen
Glucose-6-P
Metabolism, GNG

Glucose

Phosphofructokinase-1 (PFK-1)

Glycolysis

F-2,6-bisP

AMP

Cholesterol esterase

Lipid metabolism Cholesterol esters

Cholesterol + 1FA

bile salts

Phospholipases

Lipid metabolism Phosphatidylcholine

Break down ester bond

Pyruvate carboxylase

GNG

Pyruvate

Oxaloacetate

Acetyl-CoA, ATP

PEP carboxylase

GNG

Oxaloacetate

PEP

GTP

Fructose-1,6-bisPase

GNG

Fructose-1,6-bisP

Fructose-6-P

Glycerol kinase

GNG

Glycerol

Glycerol-3-P

ATP

G-3-P dehydrogenase

GNG

Glycerol-3-P

DHAP

NAD

-ketothiolase

Ketone Bodies

Acetyl-CoA

Acetoacetyl-CoA

HMG-CoA synthase

Ketone Bodies

Acetoacetyl-CoA

HMG-CoA

HMG-CoA lyase

Ketone Bodies

HMG-CoA

Acetoacetate

F-6-P

Cofactors

biotin
insulin

-ketoacyl-CoA transferase

Ketone Bodies

Acetoacetate

Acetoacetyl-CoA

Deficiency

Notes

only in liver
rate-limiting
enzyme in all
tissues

Chronic lactic
acidoses,
neurological defects

Essential fructosuria
Fructose intolerance,
inhibition of
glycolysis & GNG

hemolysis induced
by oxidative stress

Deficiency

Notes

important as a
marker of liver
function

Maple Syrup Urine

Disease

PKU
PKU-II, III
Homocysteinuria

Deficiency

Notes

McArdle's Disease

Von Gierke

Not present in
muscle; only
liver

rate-limiting

Liver doesn't
have this, so
won't use
ketone bodies