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Von Gierke Disease

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disulfidebond007's version from 2016-08-14 20:01

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Question Answer
What enzyme is deficient?G6phosphotase
How do they present?Life threatening hypoglycemia, seizures, excess fat in cheeks poor growth
Why is pyruvate elevated in Von Gierke?Increased glycolysis leads to saturation of PC and PDHC
What does Maylonyl Co A do?Inhibits CPT1
What does CPT 1 doBeta oxidation
Why Hyperlipidema?High level of pyruvate, high levels of TCA priming, citrate accumulates and is exported to cytosol, increased maylonyl Co a leads to lipogensis
HSL activity? High
What results in high HSL activity?increased lipolysis and more release of FFAs
What happens when FFAs are released?get repacked as VLDLs
Why is alanine increased?catabolism of branch chained AA in muscle cells
What does ALT do?ALT is in liver and converts alanine into pyruvate
What happens when Alanine gets converted to Pyruvate?Gets converted to lactate
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