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18 Cards in this Set
- Front
- Back
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Glucose 6-P Dehydrogenase Deficiency
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-Arise upon administrating antimalarials or sulfa antibiotics
-Patients have hemolytic anemia -Cells do not reduce enough NADP+ to maintain glutathione in its reduced state for their cell membrane. |
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Purpose of the PPP?
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-NADPH Production (reducing power)
-Production of ribose-5-P to make DNA and RNA |
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PPP overall pathway
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1-glucose-6-P + 2NADP + 2H2O ---> 2 NADPH (reduced) + Ribose 5-P + CO2
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1. Glucose-6-P ---> 6-Phosphogluconolactone (6-PGL)
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Glucose-6-P Dehydrogenase + NADPH
-First committed step -Regulated by NADPH |
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2. 6-Phosphogluconolactone (6-PGL) --->6-phosphogluconate
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gluconolactonase
H2O -converts cyclic structure to linear structure |
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3. 6-Phosphogluconate ---> Ribulose-5-Phosphate
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6-Phosphogluconate Dehydrogenase + NADPH + CO2
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4. Ribulose-5-P ---> Ribose-5-P
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Ribulose-5-P isomerase
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If need to make NADPH + Ribose-5-P
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-Do oxidative stage only
-Use ribose 5-P for DNA, RNA, NADPH |
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If need to make NADPH only
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Use whole pathway (oxidative and nonoxidative stage)
-Swap Carbons using Transketolases and Transaldolases to get glyceraldehyde 3-P and 2 moles of Fructose 6-P -Feed to the top to produce NADPH |
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NADPH + energy
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Whole pathway + glycolysis
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Ribose-5-P only
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-Runs in reverse
-Take Glyceraldehyde-3-P and Fructose-6-P to produce Ribose-5-P using TA and TK |
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Transketolase (TK)
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Take 2 carbons from Ketose to Aldose
-cofactor: Thiamine PP |
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Transaldolase (TA)
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3 carbons from ketose to aldose
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Glucose-6-P dehydrogenase deficiency
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-Oxidative stress (antimalarial drugs, infection, flava beans), hemolytic anemia
-Need NADPH to give reduced glutothione which keeps Hb reduced and rbc membrane intact |
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Thiamine Deficiency (beri beri)
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-Neurological problems, cardiac damage
-therefore problem with transketolase |
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Wernicke-Korsakoff Syndrome
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-Genetic defect + thiamine deficiency
-problems with transketolase -Neurological symptons + Beri Beri-like symptoms |
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Drug detoxification in liver
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-Require NADPH for cytochhrom P-450
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Phagocytosis
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Defense against bacteria
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