70 SEC TION II BIOCHEMISTRY `

BIOCHEMISTRY—METABOLISM AGP 2019

POLYOL PATHWAY
Summary of pathways

Galactose metabolism
Galactokinase (mild galactosemia) Galactose B Requires biotin cofactor
Galactose-1-phosphate GALACTOKINASE T Requires thiamine cofactor (TPP)
uridyltransferase Glycolysis # Irreversible, important point of regulation
(severe galactosemia) Galactose-1-phosphate Glucose [cytoplasm]
Glycogen GALACTOSE 1 HEXO
Hexokinase/glucokinase PHOSPH UDT GLUCOSE 6 KINASE
PHOSPHATASE HMP shunt
Glucose-6-phosphatase [cytoplasm]
UDP-glucose Glucose-1-phosphate Glucose-6-phosphate 6-phosphogluconolactone
(von Gierke disease) G6PD
FRUCTOSE 1,6
Glucose-6-phosphate Glycogenesis / glycogenolysis BISPHOSPHATASE
dehydrogenase Fructose-6-phosphate Ribulose-5-phosphate
TRANSKETOLASE
T
Transketolase Gluconeogenesis PFK 1
[mitochondria—>cytoplasm]
Phosphofructokinase-1 Fructose-1,6-bisphosphate Fructose metabolism
ALDOLASE B
Fructose-1,6-bisphosphatase FRUCTOKINASE

Fructokinase (essential fructosuria) *2C Glyceraldehyde-3-P DHAP Fructose-1-phosphate Fructose

Aldolase B (fructose intolerance) triose ALDOLASE B
Rapaport Luebering Biphosphoglycerate mutase
phosphate
Aldolase B (liver), A (muscle) Glycolytic Shunt 1,3-bisphosphoglycerate isomeraseGlyceraldehyde
Triose phosphate isomerase 2,3-bisphosphoglycerate

3-phosphoglycerate Glycerol Lipid metabolism

Pyruvate kinase [cytoplasm]
Pyruvate dehydrogenase Lipogenesis
2-phosphoglycerate Triglycerides
Pyruvate carboxylase
PEP carboxykinase
Phosphoenolpyruvate (PEP) Fatty acids
Citrate synthase PYRUVATE
PEP KINASE Cholesterol Synthesis
Isocitrate dehydrogenase CARBOXY
KINASE Alanine Pyruvate Lactate Cholesterol
NAD Malonyl-CoA
α-ketoglutarate dehydrogenase TPYRUVATE DHD
PYRUVATE B
CARBOXYLASE NADH2 ACETYL CoA HMG-CoA
Ornithine transcarbamylase Acetyl-CoA REDUCTASE Mevalonate
+
B CARBOXYLASE
Propionyl-CoA carboxylase Acetoacetyl-CoA HMG-CoA
HMG-CoA
HMG-CoA reductase
Citrate SYNTHASE = Acetoacetate Acetone

Oxaloacetate CITRATE
NH3 + CO2 Aspartate SYNTHASE
NADH2
Citrulline ARG Isocitrate β-hydroxybutyrate
CPS I ORNITHINE
SYNTHETASE ISOCITRATE
TRANSCARBAMYLASE [mitochondria]
Carbamoyl phosphate Argininosuccinate
NAD DHD NAD Ketogenesis
Malate TCA cycle
Ornithine ARG [mitochondria] CO2 NADH2 Odd-chain fatty acids,
LYASE α-ketoglutarate branched-chain amino acids,
[liver mitochondria] Urea cycle a-KETOGLUTARATE methionine, and threonine
[liver cytosol] Fumarate
CO2 T DHD
Arginine SUCCINATE DHD
ARGINASE
Succinate Succinyl-CoA Methylmalonyl-CoA Propionyl-CoA
Urea FADH2 B
FAD
B12 Propionyl CoA
GDP Propionyl CoA CARBOXYLASE
H2O GTP Protein metabolism
MUTASE
Heme Synthesis
Activation of Ketones
FATTY ACID SYNTHESIS / BETA OXIDATION
KETOGENESIS

CORI CYCLE
ALA synthase

Zinc containing