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17 Cards in this Set

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Purpose of RBC Metabolism
Keep a reduced environment and E metabolism
Ox + Hb--> HbO2--> O2- + MetHb
O2- Bad bc superoxide anion
MetHb Bad bc does not bind O2
Met Hb (Fe+3) ---> Hb (Fe+2)
Met Hb reductase
NADH---> NAD+ from glycolysis as GAPDH ---> 1,3 diPglycerate
O2- --->O2
Superoxide dismutase
Produce peroxide bad!
2 GSH ---> GSSG
Glutathione peroxidase
GSSG--->2GSH
Glutathione Reductase
NADPH--->NADP+ from PPP from Glc 6PDH & 6-Pgluconate DH
Glucose 6-P dehydrogenase deficiency
Low NADPH, NADH
Glutathione will be oxidized ---> GSSG
Peroxide & MetHb accumulates
What happens when peroxide accumulates?
Membrane damage
What happens when MetHb increases?
Forms granules called Heinz bodies--->distort RBC shape-->RBC lysis--->Hemolytic anemia
What other cmpds can change the reduced environment?
Anti-malarial drugs bc they accept electrons from NADPH/NADH
Effect of anti-malarial drugs
accept e from NADPH/NADH ---> cannot maintain reduced environment---> high GSSG, MetHb--->Heinz bodies form--->distort RBC--->lysis
Pyruvate Kinase deficiency (PK)
PEP, glycerate 2-P, glycerate 3-P accumulate upstream

Pyruvate, lactate, ATP lower downstream

No ATP--->needed for Na/K ATPase--->maintain plasma membrane--->cells lyse
Hemoglobinopathies
Abnormalities in globin polypeptides
1. Change in aa sequence of globin
2. Imbalance of alpha or beta chains of Hb
ie: alpha/beta-thalassemia
Sickle Cell Anemia
aa change from glutamic acid (negatively charged) GAG to Valine (neutral) GUG
When does RBC turn sickle?
Hydrophobic "patch" in oxy/deoxy HbS matches with complementary "groove"--->ppt-->distort RBC shape-->sickle shape
When can sickle cell anemia occur?
Acidosis
Exercise-high H+ and CO2
High Altitude-low pO2
Hypoxia
Hemoglobin M
aa changes from Tyr to Hist
Fe+3 becomes more stable than Fe+2
Fe+3 is MetHb and does not bind O2--> lysis