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ATP
Hexokinase (low Km) Glucokinase (high Km)
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
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
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