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12 Cards in this Set
- Front
- Back
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Why is the PPP important?
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- it is an alternative pathway for Glucose-6-P
- This pathway involves the most COMMON inherited Metabolic Defect - makes ribose-5-P needed for nucleotide synth |
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Why do we need NADPH?
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REDUCING FACTOR!!!
- reduction of glutathione - FA and Cholesterol Synth - Phagocytosis - NO synthersis - Cytochrome p450 system |
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What does G6PDH do?
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Reduces Glucose-6P to Ribulose 5-P, also produces lots of NADPH
- Part of the IRREVERSIBLE OXIDATIVE RXNS |
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What is Transketolase? What type of rxns is it involved with?
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- Converts Ribose to Glyceraldehyde-3-P which will go into Glycolysis
- requires TPP - involved with REVERSIBLE NONOXIDATIVE rxns - |
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What is made in the Oxidative Reactions of PPP?
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NADPH
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What is the rate limiting step of PPP?
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G6PDH
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What is the product inhibitor of G6PDH?
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NADPH
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What upregulates G6PDH expression?
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Insulin
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What is a result of G6PDH deficiency?
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- most common disease causing enzyme deficiency
- causes HEMOLYTIC ANEMIA - RBC are most affected bc PPP is the only means of making NADPH ---> hence, no glutathione to protect Hb |
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What are Sx of G6PD deficiency?
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- Oxidative Stress
- Oxidizing drugs - FAVA BEANS |
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What are Heinz Bodies?
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blobs of denatured MetHb
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What reduces glutathion after if becomes oxidized?
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Glutathione DH
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