GLYCOLYSIS

ATP
Hexokinase (low Km) Glucokinase (high Km)

G6PDHase Deficiency Glucose

• • • •

X-Linked disorder (4x106 affected) Associated w/fava beans GSSG + NAPDH  GSH + NADP+ ↓ GSH  ↑ oxidative damage
Hexose monophosphate

ADP Glycogen
G-6-P

NADP+
G6PDHase

ATP
Phosphofructokinase-1 (PFK-1)
*COMMITTED Step

F-6-P

NADPH Pentose monophosphate

ADP

F-1,6-bP

G-3-P
Enzyme Regulation • Hexokinase: All cells; Low Km • Glucokinase: Hepatocytes; High Km • PFK-1: COMMITTED STEP

Continued…

DHAP

F-1-P

Fructose

Continued…

G-3-P

Arsenic Poisoning

PO43- + NAD+
Glyceraldehyde-3-Phosphate (G3P) Dehydrogenase

NADH 1,3-bPG

• AsO43- analogous to PO43• Replaces PO43- in GAPDHase rxn • Decreases ATP synthesis

ADP
Phosphoglycerate (PG) Kinase

2,3-bPG
*Hb-O2 Regulator

ATP 3-PG

PO43_ Hypoglycemia, acidemia, and NaF

2-PG
Enolase

Pyr-Kinase Deficiency • Autosomal recessive • Hemolytic anemia b/c RBCs dependent on anaerobic respiration ADP
Pyruvate Kinase

PEP

• RBCs anaerobically dependent • False hypoglycemia & acidemia w/blood draws common • NaF added to blood sample… • Enolase inhibited by F- ion

ATP Pyruvate

GLYCOLYSIS

Fates of Pyruvate
Product Lactate Acetyl-CoA Oxaloacetate Alanine Enzyme Lactate dehydrogenase Pyruvate dehydrogenase complex Pyruvate carboxylase Alanine transaminase Function Anaerobic pathway; regenerate NAD+ TCA Cycle entry TCA Cycle replenishment Amino-group carrier (to liver)

Pyruvate Dehydrogenase Complex (PDC) • 3 Enzymes • 5 Co-Factors: Vitamin B1,2,3,5 and Lipoic acid

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